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> Disorders > Disease or Syndrome > Hepatolenticular Degeneration

Hepatolenticular Degeneration

Hepatolenticular Degeneration is a rare genetic disorder characterized by the accumulation of copper in various organs, particularly the liver and brain.
This condition, also known as Wilson's Disease, can lead to liver damage, neurological impairments, and psychiatric symptoms if left untreated.
Early diagnosis and prompt treatment are essential to manage the condition and prevent long-term complications.
PubCompare.ai's AI-driven platform can assist researchers in exploring the latest protocols, pre-prints, and patents related to Hepatolenticular Degeneration, enabling them to identify the most effective approaches and improve research reproducibility and accuracy.

Most cited protocols related to «Hepatolenticular Degeneration»

1
Optimized Fibrosis Scoring for Older NAFLD Patients
This study included consecutive patients with biopsy-proven NAFLD who attended specialist fatty liver clinics at the Freeman Hospital, Newcastle upon Tyne, UK; Addenbrooke's Hospital, Cambridge, UK; Antwerp University Hospital, Edegem, Belgium; and Pitié-Salpêtrière Hospital, Paris, France. These formed the initial ascertainment cohort. Liver biopsies were conducted as per routine clinical care for the investigation of abnormal liver function tests (raised ALT, AST, or gamma-glutamyl transferase) or to stage disease severity in patients with radiological evidence of fatty liver. Clinical and laboratory data were collected prospectively from the time of liver biopsy. Patients with evidence of other liver disease (autoimmune hepatitis, viral hepatitis, drug induced liver injury, hemochromatosis, cholestatic liver disease, or Wilson's disease) were excluded. In addition, subjects consuming excessive amounts of alcohol (alcohol intake >20 g/day for women; >30 g/day for men) at the time of biopsy or in the past were excluded. Patients with incomplete data to calculate all the non-invasive scores were excluded.
Relevant clinical details, including gender, age, weight, and height, were obtained at the time of biopsy. The body mass index was calculated by the formula: weight (kg)/height (m)2. Patients were identified as having diabetes if they had been diagnosed with diabetes according to the 2004 American Diabetes Association criteria or if they were taking an oral hypoglycemic drug or insulin (23 (link)).
Percutaneous liver biopsies were performed as per unit protocol at the sites and were assessed by an experienced local hepatopathologist. Patients with liver biopsies specimens <15 mm in length were excluded. Histological scoring was performed according to the non-alcoholic steatohepatitis (NASH) Clinical Research Network criteria (24 (link)). The NAFLD activity score was graded from 0 to 8, including scores for steatosis (0–3), lobular inflammation (0–3), and hepatocellular ballooning (0–2). NASH was defined as steatosis with hepatocyte ballooning and inflammation ± fibrosis (25 (link)). Fibrosis was staged from F0 to F4 (24 (link)). Patients with stage F3 or F4 fibrosis were considered to have advanced fibrosis.
The AST/ALT ratio, FIB-4, and NFS were calculated from blood tests taken at the time of liver biopsy as previously described (16 (link), 26 (link), 27 (link)). Details of the formulas and cutoffs for the tests under investigation are shown in Table 1. Previously published cutoffs were used to exclude and diagnose advanced fibrosis for each score (15 (link), 16 (link), 18 (link), 19 (link))
To validate new cutoffs for the NFS and FIB-4 score optimized for use in older patients (aged ≥65 years) that were derived in the initial ascertainment cohort, anonymized biochemical, histological, and anthropometric data were collected from a separate group of histologically characterized patients from the EPoS/EASL European NAFLD Registry. The “European NAFLD Registry” was established during the EU FP7 FLIP project (2010-) and is now maintained by the EU H2020 EPoS (Elucidating Pathways of Steatohepatitis) consortium to facilitate collaborative research into NAFLD. It is the largest multi-national registry of patients with histologically characterized NAFLD. These patients had data collected according to the same methodology as the main cohort.
All statistical analyses were performed using the SPSS software version 22.0 (SPSS, Chicago, IL). Continuous normally distributed variables were represented as mean ± s.d. Categorical and non-normal variables were summarized as median and range. Chi squared tests were used to determine the distribution of categorical variables between groups. To compare the means of normally distributed variables between groups, the Student's t-test or analysis of variance test was performed. To determine differences between groups for continuous non-normally distributed variables, medians were compared using the Mann–Whitney U-test. The diagnostic performance of the non-invasive tests was assessed by receiver operating characteristic (ROC) curve analysis. The area under the ROC (AUROC) was used as an index to compare the accuracy of tests. The sensitivity, specificity, positive predictive values (PPVs), negative predictive values (NPVs), positive likelihood ratios (LR +ve), and negative likelihood ratios (LR −ve) for relevant cutoffs were also displayed. In order to assess changes in sensitivity and specificity of the tests with age, plots of sensitivity and specificity in different age groups were displayed graphically. New cutoffs for the FIB-4 and NFS were derived for ≥65-year-old patients by taking the point on the ROC where the combined value of sensitivity and specificity was the highest. As the prevalence of advanced fibrosis can vary in different populations, the PPVs and NPVs for the new cutoffs were displayed at advanced fibrosis prevalence rates of 5, 10, 20, 30, and 40%. A P value of <0.05 was considered significant.
McPherson S., Hardy T., Dufour J.F., Petta S., Romero-Gomez M., Allison M., Oliveira C.P., Francque S., Van Gaal L., Schattenberg J.M., Tiniakos D., Burt A., Bugianesi E., Ratziu V., Day C.P, & Anstee Q.M. (2016). Age as a Confounding Factor for the Accurate Non-Invasive Diagnosis of Advanced NAFLD Fibrosis. The American Journal of Gastroenterology, 112(5), 740-751.
Publication 2016
Age Groups Autoimmune Chronic Hepatitis Biopsy Cholestasis Diabetes Mellitus Diagnosis Diet, Formula Drug-Induced Liver Disease Erythropoietin Ethanol Europeans Fatty Liver Fibrosis gamma-Glutamyl Transpeptidase Gender Hematologic Tests Hemochromatosis Hepatitis Viruses Hepatocyte Hepatolenticular Degeneration Hypersensitivity Hypoglycemic Agents Index, Body Mass Inflammation Insulin Liver Liver Diseases Liver Function Tests Non-alcoholic Fatty Liver Disease Nonalcoholic Steatohepatitis Patients Population Group Specialists Steatohepatitis Tests, Diagnostic Woman X-Rays, Diagnostic
2
NAFLD Inclusion Criteria in NIH CRN Studies

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Publication 2009
Adult Alcohol Use Disorder Antibodies, Antinuclear Autoimmune Chronic Hepatitis Child Ethanol Fibrosis Hemochromatosis Hepatitis B Hepatolenticular Degeneration Hypergammaglobulinemia Liver Diseases Metformin Necrosis Non-alcoholic Fatty Liver Disease Nonalcoholic Steatohepatitis Pioglitazone Placebos Primary Biliary Cholangitis SERPINA5 protein, human TimeLine Vitamin E Woman
3
Diagnosing NAFLD in Obese Children

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Publication 2010
Amiodarone Asparaginase Celiac Disease Child Cystic Fibrosis Diabetes Mellitus, Insulin-Dependent Disease, Chronic Fatty Liver Glucocorticoids Hepatitis C virus Hepatolenticular Degeneration Liver Non-alcoholic Fatty Liver Disease Obesity Pharmaceutical Preparations Serum Valproic Acid
4
Childhood NAFLD Diagnosis and Assessment
A total of 208 consecutive patients diagnosed with NAFLD seen at Bambino Gesù Children’s Hospital from January 2005 to January 2010 were included in the study. The study was approved by the Ethics Committee of the Bambino Gesù Children’s Hospital and Research Institute, Rome, Italy. Informed consent was obtained from each patient or responsible guardian.
Inclusion criteria were liver biopsy consistent with the diagnosis of NAFLD (8 (link), 9 (link)). Exclusion criteria were the presence of hepatic virus infections, alcohol consumption (≥140gm/week), history of parenteral nutrition, and use of drugs known to induce steatosis (e.g. valproate, amiodarone or prednisone) or to affect body weight and carbohydrate metabolism. Autoimmune liver disease, metabolic liver disease, Wilson’s disease, and α-1-antitrypsin-associated liver disease were ruled out using standard clinical, laboratory and histological criteria.
The body mass index (BMI) and BMI Z-score were calculated (10 (link), 11 (link)). Metabolic syndrome (MS) was defined as the presence of ≥ 3 of the following 5 criteria (12 (link)): abdominal obesity (defined by waist circumference ≥ 90th percentile for age) (13 (link)); hypertriglyceridemia as TG > 95th percentile for age, gender and race (14 (link)); low HDL cholesterol as concentrations < 5th percentile for age and sex (14 (link)); elevated blood pressure (BP) as systolic or diastolic BP > 95th percentile for age and sex (15 (link)); and impaired fasting glucose or known type 2 diabetes mellitus (16 (link), 17 (link)). The degrees of insulin resistance (IR) and sensitivity were determined by the homeostasis model assessment for insulin resistance (HOMA-IR) using the formula: insulin resistance = (insulin × glucose)/22.5, by the insulin sensitivity index (ISI) derived from oral glucose tolerance test using the formula: ISI = [10,000/square root of (fasting glucose × fasting insulin) × (mean glucose × mean insulin during OGTT) and by the quantitative insulin sensitivity check index (QUICKI) using the formula: insulin sensitivity = 1/(log of fasting insulin + log of fasting glucose) (18 (link)-20 (link)).
Shannon A., Alkhouri N., Carter-Kent C., Monti L., Devito R., Lopez R., Feldstein A.E, & Nobili V. (2011). Ultrasonographic Quantitative Estimation of Hepatic Steatosis in Children with Nonalcoholic Fatty Liver Disease (NAFLD). Journal of pediatric gastroenterology and nutrition, 53(2), 190-195.
Publication 2011
Amiodarone Autoimmune Diseases Biopsy Body Weight Carbohydrate Metabolism Child Clinical Laboratory Services Diabetes Mellitus, Non-Insulin-Dependent Diagnosis Ethics Committees, Clinical Glucose Hepatolenticular Degeneration High Density Lipoprotein Cholesterol Homeostasis Hypersensitivity Hypertriglyceridemia Index, Body Mass Insulin Insulin Resistance Insulin Sensitivity Legal Guardians Liver Liver Diseases Metabolic Diseases Metabolic Syndrome X Non-alcoholic Fatty Liver Disease Oral Glucose Tolerance Test Parenteral Nutrition Patients Pharmaceutical Preparations Plant Roots Prednisone Pressure, Diastolic SERPINA1 protein, human Steatohepatitis Systolic Pressure Valproate Virus Diseases Vision Waist Circumference
5
Comprehensive Evaluation of NAFLD Patients
A total of 576 patients with well-characterized and liver-biopsy-confirmed NAFLD between 2002 and 2008 were enrolled from the Japan Study Group of NAFLD (JSG-NAFLD), which includes nine hepatology centers in Japan: Center for Digestive and Liver Diseases, Nara City Hospital; Division of Gastroenterology, Yokohama City University Graduate School of Medicine; Department of Medicine and Molecular Science, Graduate School of Biomedical Sciences, Hiroshima University; Department of Gastroenterology and Hepatology, Kochi Medical School; Department of Internal Medicine, Saga Medical School, Saga University; Department of Hepatology, Graduate School of Medicine, Osaka City University; Department of Gastroenterology and Hepatology, Kyoto Prefectural University of Medicine; Division of Gastroenterology and Hematology/Oncology, Department of Medicine, Asahikawa Medical College; and Hepatology Center, Saiseikai Suita Hospital. All patients were also involved in the previous JSG-NAFLD study [25 (link)].
The diagnosis of NAFLD was based on the following criteria: (1) liver biopsy showing steatosis in at least 5% of hepatocytes [26 (link)]; and (2) appropriate exclusion of liver diseases of other etiologies, including viral hepatitis, autoimmune hepatitis, drug-induced liver disease, primary biliary cirrhosis, biliary obstruction, hemochromatosis, Wilson's disease, or α-1-antitrypsin- deficiency-associated liver disease. Patients who consumed > 20 g alcohol per day and patients with evidence of decompensated LC or HCC were excluded. Written informed consent was obtained from all patients at the time of liver biopsy, and the study was conducted in accordance with the Helsinki Declaration [27 (link)]. The study protocol was approved by the ethical committee of Nara City Hospital in Nara, Japan.
Sumida Y., Yoneda M., Hyogo H., Itoh Y., Ono M., Fujii H., Eguchi Y., Suzuki Y., Aoki N., Kanemasa K., Fujita K., Chayama K., Saibara T., Kawada N., Fujimoto K., Kohgo Y., Yoshikawa T, & Okanoue T. (2012). Validation of the FIB4 index in a Japanese nonalcoholic fatty liver disease population. BMC Gastroenterology, 12, 2.
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Publication 2012
alpha 1-Antitrypsin Deficiency Autoimmune Chronic Hepatitis Biopsy Cholestasis Diagnosis Digestive System Drug-Induced Liver Disease Ethanol Hemochromatosis Hepatitis Viruses Hepatocyte Hepatolenticular Degeneration Liver Liver Diseases Neoplasms Non-alcoholic Fatty Liver Disease Patients Pharmaceutical Preparations Primary Biliary Cholangitis Steatohepatitis

Most recents protocols related to «Hepatolenticular Degeneration»

1
Pediatric Liver Transplant Indications
Diagnosis groups are described in Table 1; they represent the top 5 indications for pediatric liver transplantation based on data from 2006 to 2016. These groups, in descending order of frequency, are as follows: biliary atresia, acute hepatic necrosis (AHN), metabolic disorders, hepatoblastoma, and autoimmune cirrhosis. Biliary atresia served as the reference group. Different variations of the same general diagnosis category were grouped together; for example, patients listed with alpha-1-antitrypsin deficiency would be placed in the same indication category (metabolic disorders) as patients listed with Wilson's disease.
Lang A., Goff C., Montgomery A., Lynn J., Kamepalli S., Goss J, & Rana A. (2023). Disparate Intent-to-Treat Outcomes for Pediatric Liver Transplantation Based on Indication. Canadian Journal of Gastroenterology & Hepatology, 2023, 2859384.
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Publication 2023
alpha 1-Antitrypsin Deficiency Biliary Atresia Diagnosis Hepatoblastoma Hepatolenticular Degeneration Liver Cirrhosis Liver Transplantations Massive Hepatic Necrosis Metabolic Diseases Patients
2
Parkinson's Disease Visuospatial Assessment
We consecutively enrolled patients who visited our study group for PD between November 2021 and September 2022. Eligible patients were those who were diagnosed with PD according to the International Parkinson and Movement Disorder Society (MDS) criteria (Postuma et al., 2015 (link)). Exclusion criteria were any neurological disorder other than PD including parkinsonism secondary to trauma or drugs, metabolic diseases, encephalitis, progressive supranuclear palsy, essential tremor, and hepatolenticular degeneration. All eligible patients underwent assessment via APP tests including DST, VOT, FRT, and VMT in the APP with raw scores recorded, at the same time, CDT, CCT, and MMSE were also evaluated as classic evaluation tools for comparison. Patients with the CDT score of 5 (Spenciere et al., 2017 (link)) and the CCT score ≥ 18 (Bu et al., 2013 (link)) were classified to no visuospatial disorder group, while patients with the CDT score < 5 or CCT score < 18 were classified to visuospatial disorder group. Information on patients’ demographic characteristics and clinical profile were collected from medical records. This study was performed in accordance with the Declaration of Helsinki and approved by the ethics committee of China-Japan friendship Hospital (2020-129-K82). All participants gave their informed consent to participate in the study in written form.
Shao X., Wang K., Zhang Y., Zhen X., Dong F., Tian H, & Yu Y. (2023). Outcome of visuospatial dysfunction assessment in patients with Parkinson’s disease using mobile application software. Frontiers in Aging Neuroscience, 15, 1108166.
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Publication 2023
Compassion Fatigue Encephalitis Essential Tremor Ethics Committees, Clinical Hepatolenticular Degeneration Metabolic Diseases Mini Mental State Examination Movement Disorders Nervous System Disorder Parkinsonian Disorders Patients Pharmaceutical Preparations Progressive Supranuclear Palsy
3
Wilson's Disease Diagnostic Protocol
Fifty patients with Wilson’s disease (29 men, 21 women, age: 33.6 ± 12.4 years, mean ± SD, duration of disease: 11.4 ± 7.1 years) were involved in the study. Diagnosis of Wilson’s disease was based on the Leipzig score [15 (link)] and only data of patients with a Leipzig score ≥ 4 were included. Liver involvement was diagnosed if any of the following were detected: aspartate aminotransferase > 50 U/l, prothrombin time > 19 s, or serum bilirubin > 34 μM/l. The diagnosis of liver cirrhosis was based on hepatosplenomegaly, abdominal ultrasound, Fibroscan and histological findings. Exclusion criteria were as follows: liver involvement of viral (hepatitis B virus surface antigen and anti-HCV positivity) or autoimmune etiology (antinuclear, anti-smooth muscle, anti-mitochondrial antibody positivity), liver cancer, and treatment with hepatotoxic drugs. No liver-transplanted patients were included. Patients were on penicillamine or trientine therapy. The study was approved by the ethics committee of Semmelweis University (Semmelweis University Regional and Institutional Committee of Science and Research Ethics, 62/2019) and complied with the guidelines of the Declaration of Helsinki. All patients gave written informed consent.
Vörös K., Márkus B., Atzél K., Szalay F., Gráf L., Németh D., Masszi T., Torzsa P, & Kalabay L. (2023). Serum fetuin-A is decreased in cirrhotic patients with Wilson’s disease. PLOS ONE, 18(3), e0282801.
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Publication 2023
Abdomen Antibodies, Anti-Idiotypic Aspartate Transaminase Bilirubin Cancer of Liver Diagnosis Ethics Committees Hepatitis B Surface Antigens Hepatitis C Antibodies Hepatolenticular Degeneration Liver Liver Cirrhosis Mitochondria Patients Penicillamine Pharmaceutical Preparations Serum Smooth Muscles Therapeutics Times, Prothrombin Trientine Ultrasonography Woman
4
Hepatobiliary Manifestations in UC Patients
This is a prospective observational study on 167 patients with hepatobiliary manifestations who underwent two-stage elective LRP with IPAA for UC from June 2013 to June 2018 at our universities’ hospitals. Inclusion criteria were all patients between 18 and 69 years; men and women with at least one hepatobiliary manifestation. In patients with UC, surgery was decided according to The European Crohn’s and Colitis Organisation guidelines on therapeutics in UC[11 (link)]. Exclusion criteria included: Alcohol abuse, severe heart failure or type II diabetes mellitus, complications or death related to LRP operation, liver toxicity of IBD-related medications, active or chronic viral hepatitis, hemochromatosis, Wilson's disease, drugs-induced steatosis (amiodarone or tamoxifen), morbid obesity or patients undergoing bariatric surgery, immunoglobulin G4-related cholangitis; human immunodeficiency virus/acquired immune deficiency syndrome; tuberculosis; secondary sclerosing cholangitis; cholangiocarcinoma; complications of advanced PSC (hepatic encephalopathy, portal hypertension, hepatorenal syndrome, or hepato-pulmonary syndrome; end-stage liver failure), hypercoagulability status (systemic lupus erythematosus, increased von Willebrand factor or increased homocysteine level), oral contraceptive pills, Grave's disease, dyslipidemia, and previous biliary tract surgery including cholecystectomy.
Habeeb T.A., Hussain A., Podda M., Cianci P., Ramshaw B., Safwat K., Amr W.M., Wasefy T., Fiad A.A., Mansour M.I., Moursi A.M., Osman G., Qasem A., Fawzy M., Alsaad M.I., Kalmoush A.E., Nassar M.S., Mustafa F.M., Badawy M.H., Hamdy A., Elbelkasi H., Mousa B., Metwalli A.E., Mawla W.A., Elaidy M.M., Baghdadi M.A, & Raafat A. (2023). Hepatobiliary manifestations following two-stages elective laparoscopic restorative proctocolectomy for patients with ulcerative colitis: A prospective observational study. World Journal of Gastrointestinal Surgery, 15(2), 234-248.
Publication 2023
167-A Abuse, Alcohol Acquired Immunodeficiency Syndrome Amiodarone Bariatric Surgery Biliary Tract Surgical Procedures Cholangiocarcinoma Cholangitis Cholecystectomy Colitis Congestive Heart Failure Contraceptives, Oral Crohn Disease Diabetes Mellitus, Non-Insulin-Dependent Dyslipidemias End Stage Liver Disease Europeans Factor VIII-Related Antigen Graves Disease Hemochromatosis Hepatic Encephalopathy Hepatitis, Chronic Hepatolenticular Degeneration Hepatopulmonary Syndrome Hepatorenal Syndrome HIV Homocysteine IgG4 Liver Lupus Erythematosus, Systemic Obesity, Morbid Patients Pharmaceutical Preparations Portal Hypertension Steatohepatitis Tamoxifen Therapeutics Thrombophilia Toxicity, Drug Tuberculosis Woman
5
Diagnostic Criteria for Hepatobiliary Conditions
PSC is progressive biliary fibrosis affecting intra and/or extrahepatic bile ducts[12 (link)] and diagnosed by laboratory tests [(cholestasis, Antineutrophil cytoplasmic antibodies (ANCA)], radiology [abdominal ultrasonography (US), abdominal computed tomography (CT), endoscopic retrograde cholangiopancreatography (ERCP), or magnetic resonance cholangiopancreatography (MRCP)], and liver biopsy. Primary biliary cholangitis (PBC) is characterized by the loss of small and medium-sized bile ducts on liver biopsy, elevated anti-mitochondrial antibodies, and altered gamma-glutamyl transferase and alkaline phosphatase (ALP) levels[13 (link)]. Non-alcoholic fatty liver disease (NAFLD) is characterized by fat storage in ≥ 5% of hepatic steatosis in the absence of concomitant liver disease (chronic viral hepatitis), use of steatosis-inducing medications (amiodarone or tamoxifen), autoimmune hepatitis, hemochromatosis, Wilson's disease, or excessive alcohol consumption[14 (link)]. Diagnosis of NAFLD was made by liver biopsies or US[15 (link)], and the severity score was previously stated[16 (link)]. Autoimmune hepatitis diagnosis based on the International Autoimmune Hepatitis Group criteria with a score of > 15 points consisting of demographic, histologic, and laboratory markers, including antinuclear antibodies with a titer of at least 1:40 and liver histology[17 (link)]. An aseptic liver abscess is diagnosed based on IBD history, US, and CT[18 (link)]. Ultrasound, colour Doppler, and/or CT scans were used to detect portal vein thrombosis.
Habeeb T.A., Hussain A., Podda M., Cianci P., Ramshaw B., Safwat K., Amr W.M., Wasefy T., Fiad A.A., Mansour M.I., Moursi A.M., Osman G., Qasem A., Fawzy M., Alsaad M.I., Kalmoush A.E., Nassar M.S., Mustafa F.M., Badawy M.H., Hamdy A., Elbelkasi H., Mousa B., Metwalli A.E., Mawla W.A., Elaidy M.M., Baghdadi M.A, & Raafat A. (2023). Hepatobiliary manifestations following two-stages elective laparoscopic restorative proctocolectomy for patients with ulcerative colitis: A prospective observational study. World Journal of Gastrointestinal Surgery, 15(2), 234-248.
Publication 2023
Abdomen Alkaline Phosphatase Amiodarone Anti-Antibodies Antibodies, Antinuclear Antineutrophil Cytoplasmic Antibodies Asepsis Autoimmune Chronic Hepatitis Bile Bile Ducts, Extrahepatic Biopsy Cholangiopancreatography, Magnetic Resonance Cholestasis Duct, Bile Endoscopic Retrograde Cholangiopancreatography Fatty Liver Fibrosis gamma-Glutamyl Transpeptidase Hemochromatosis Hepatic Duct Hepatitis, Chronic Hepatolenticular Degeneration Liver Liver Abscess Liver Diseases Mitochondria Non-alcoholic Fatty Liver Disease Pharmaceutical Preparations Primary Biliary Cholangitis Radiography Radionuclide Imaging Steatohepatitis Tamoxifen Thrombosis Ultrasonography Veins, Portal Venous Thrombosis X-Ray Computed Tomography

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1
Cobas ampliprep cobas taqman hcv test by Roche
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The COBAS AmpliPrep/COBAS TaqMan HCV Test is a diagnostic laboratory equipment product designed for the detection and quantification of Hepatitis C Virus (HCV) RNA in human serum or plasma samples. It utilizes real-time PCR technology to provide automated sample preparation and amplification, delivering reliable and accurate results.
2
Hepg2 cell by American Type Culture Collection
Sourced in United States, China, United Kingdom, Germany, Japan, Canada
HepG2 cells are a well-established human hepatocellular carcinoma cell line derived from the liver tissue of a 15-year-old Caucasian male. They are commonly used in cell-based assays and research studies related to liver function, metabolism, and toxicology.
3
3.5 mhz probe by Toshiba
Sourced in Japan
The 3.5-MHz probe is a laboratory equipment component designed for various scientific and research applications. It operates at a frequency of 3.5 megahertz and serves as a core function for data acquisition and signal processing tasks within the laboratory setting. The detailed specifications and intended use cases for this product are not available.
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Mtesr1 by STEMCELL
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MTeSR1 is a complete serum-free culture medium formulated for the maintenance of undifferentiated human embryonic stem cells (hESCs) and human induced pluripotent stem cells (hiPSCs) in feeder-free conditions.
5
Trientine hydrochloride by Merck Group
Sourced in United States
Trientine hydrochloride is a chemical compound used in the treatment of Wilson's disease, a rare genetic disorder that causes the accumulation of copper in various organs. It acts as a chelating agent, which means it binds to and removes excess copper from the body.
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Jem 1200ex by JEOL
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The JEM-1200EX is a transmission electron microscope (TEM) manufactured by JEOL. It is designed to provide high-resolution imaging and analysis of samples at the nanoscale. The core function of the JEM-1200EX is to magnify and focus a beam of electrons to create an image of the internal structure of a specimen.
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More about "Hepatolenticular Degeneration"

Hepatolenticular Degeneration, also known as Wilson's Disease, is a rare genetic disorder characterized by the accumulation of copper in various organs, particularly the liver and brain.
This condition can lead to liver damage, neurological impairments, and psychiatric symptoms if left untreated.
Early diagnosis and prompt treatment are essential to manage the condition and prevent long-term complications.
Researchers can explore the latest protocols, pre-prints, and patents related to Hepatolenticular Degeneration using PubCompare.ai's AI-driven platform.
This innovative tool can assist in identifying the most effective approaches and improving research reproducibility and accuracy.
The condition is associated with several related terms, including Hepatic Cirrhosis, Extrapyramidal Symptoms, and Kayser-Fleischer Rings.
Abbreviations commonly used include HLD and WD.
Subtopics within Hepatolenticular Degeneration include Copper Metabolism, Neuropsychiatric Manifestations, and Chelation Therapy.
To further enrich the understanding of this topic, researchers may find the following information relevant: - COBAS AmpliPrep/COBAS TaqMan HCV Test: A diagnostic tool used to detect and quantify hepatitis C virus (HCV) in patient samples. - HepG2 cells: A human liver cancer cell line often used in research related to liver diseases, including Hepatolenticular Degeneration. - 3.5-MHz probe: A type of ultrasound probe used to image the liver and detect abnormalities. - MTeSR1: A defined, serum-free medium used for the culture of human embryonic stem cells, which may have applications in regenerative medicine for liver disease. - Trientine hydrochloride: A copper-chelating agent used as a treatment for Wilson's Disease. - JEM-1200EX: A type of transmission electron microscope used to study cellular structures, which could aid in understanding the pathophysiology of Hepatolenticular Degeneration. - DNA Blood Midi Kit: A DNA extraction kit used to obtain high-quality genomic DNA from blood samples, which may be useful for genetic analysis in Hepatolenticular Degeneration. - DL-α-tocopheryl acetate: A form of vitamin E that has been investigated for its potential therapeutic effects in liver diseases. - Matrigel: A basement membrane extract used as a cell culture substrate, which could be relevant for liver cell culture models. - GeneScan 500 LIZ size standard: A DNA size standard used in genetic analysis, which may be employed in studies of the genetic basis of Hepatolenticular Degeneration.
Rearchers can leverge these insights to expand their understanding and exploration of Hepatolenticular Degeneration, ultimately leading to more effective treatments and improved patient outcomes.