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Mice, Inbred mdx

Inbred mdx mice are a valuable model for studying Duchenne muscular dystrophy, a genetic disorder characterized by progressive muscle degeneration.
These mice harbor a mutation in the dystrophin gene, resulting in the absence of functional dystrophin protein, similar to the human condition.
Researchers utilize inbred mdx mice to investigate the pathogenesis of the disease, evaluate potential therapies, and develop new treatment strategies.
PubCompare.ai's AI-driven platform can help optimize your mdx mouse research by identifying the most reproducible and accurate protocols from published literature, preprints, and patents.
With intelligent comparisons, you can easily locate the best products and procedures to advance your studies and accelerate the development of new therapies for Duchenne muscular dystrophy.

Most cited protocols related to «Mice, Inbred mdx»

Assessing Donor-Derived Muscle Fibers

Cited 10 times

Engrafted muscles were removed 4 weeks after cell injection, and when notexin was used, 1 week after myotoxin injection. Muscles that had been grafted with 3F-nLacZ-2E satellite cells were frozen in isopentane cooled in liquid nitrogen. Seven-micrometer transverse cryosections were collected at 100-μm intervals from the entire muscle. Sections were X-gal stained as previously described [27 (link)] and, only if positive signal was detected (thus under-reporting the amount of donor muscle, but avoiding inclusion of host, revertant fibers in our quantification of donor-derived muscle fibers), serial sections were immunostained using primary antibodies rabbit anti-dystrophin (P7) and, in some experiments, with mouse anti-neonatal myosin (BF34; Developmental Studies Hybridoma Bank) after blocking with 10% goat serum.
Collagen VI (Abcam, Cambridge, U.K., http://www.abcam.com) and laminin (Sigma) antibodies were used in representative sections of TA muscle from 3-week-old (n = 4) and 9-month-old (n = 3) mdx-nude mice and of 12- (n = 3) and 24-month-old (n = 2) mdx mice for detection of increase in connective tissue indicative of fibrosis Secondary antibodies used were as follows: Alexa Fluor 488-conjugated goat anti-mouse Ig (Molecular Probes) and Alexa Fluor 594-conjugated goat anti-rabbit Ig (Molecular Probes).
Muscles that had been grafted with Myf5^nLacZ/+ satellite cells were fixed in paraformaldehyde and X-gal stained as described elsewhere [3 (link), 27 (link)].

Boldrin L., Zammit P.S., Muntoni F, & Morgan J.E. (2009). Mature Adult Dystrophic Mouse Muscle Environment Does Not Impede Efficient Engrafted Satellite Cell Regeneration and Self-Renewal. Stem cells (Dayton, Ohio), 27(10), 10.1002/stem.162.

Publication 2009

5-bromo-4-chloro-3-indolyl beta-galactoside Alexa594 alexa fluor 488 Antibodies Cells Collagen Connective Tissue Cryoultramicrotomy Dystrophin Fibrosis Freezing Goat Hybridomas Infant, Newborn isopentane Laminin Mice, Inbred mdx Mice, Nude Molecular Probes Mus Muscle Tissue Myosin ATPase myotoxin A Nitrogen notexin paraform Rabbits Serum Tissue Donors

Generation of Double Mutant Mice

Cited 8 times

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Publication 2010

Animals Animals, Laboratory Immunologic Deficiency Syndromes Mice, Inbred mdx Mice, Inbred NOD Mice, Laboratory SCID Mice

Transplantation of Pax7-ZsGreen Muscle Stem Cells

Cited 7 times

For analyses of activated satellite cells, left hind limbs of Pax7-ZsGreen mice were injured with 500 µl in multiple injections of 1 µM cardiotoxin (Naja nigricollis; Calbiochem), and 3 days postinjury, analysis was performed. For transplantation, dystrophin-deficient mice (mdx^−/− ; Jackson Laboratory, Bar Harbor, ME, http://www.jax.org) immunosuppressed with 500 µg/ml tacrolimus (ChemPacific, Baltimore, http://www.chempacific.com) were used. Tibialis anterior (TA) muscles of mdx^−/− mice were injured by 100 µl of cardiotoxin 24 hours before transplantation. Pax7-ZsGreen⁺ cells expanded for 8 days in culture were resuspended in PBS (2,000 or 10,000 cells in 10 µl of PBS) and injected into injured TA muscle [6 (link)]. Three weeks after transplantation TA muscles were harvested and quick-frozen in Tissue-Tek OCT Compound (Sakura Finetek, Torrance, CA, http://www.sakura.com) compound and stored at −80°C. Analyses and quantification of the dystrophin-positive fibers were done as described previously [6 (link)] by counting the dystrophin-positive muscle fibers in representative transverse sections from the mid-belly (n = 4) of the TA muscle.

BOSNAKOVSKI D., XU Z., LI W., THET S., CLEAVER O., PERLINGEIRO R.C, & KYBA M. (2008). Prospective Isolation of Skeletal Muscle Stem Cells with a Pax7 Reporter. Stem cells (Dayton, Ohio), 26(12), 3194-3204.

Publication 2008

Cardiotoxins Cells DMD protein, human Freezing Mice, Inbred mdx Mus Muscle Tissue Naja PAX7 protein, human Satellite Cell, Muscle Tacrolimus Tibial Muscle, Anterior Tissues Transplantation

Isolation of Primary Muscle Cells from mdx Mice

Cited 7 times

Primary muscle cells were isolated from 3-wk-old mdx mice (C57BL/10ScSn mdx/mdx, The Jackson Laboratory) using a technique previously described (Rando and Blau 1994; Qu et al. 1998). The isolated cells were then suspended in the growth medium (DME supplemented with 10% FBS, 10% horse serum, 0.5% chick embryo extract, and 2% penicillin/streptomycin). The cells were then preplated in collagen-coated flasks (Rando and Blau 1994; Qu et al. 1998). After ∼1 h, the supernatant was withdrawn from the flask and replated in a fresh collagen-coated flask. The cells that adhered rapidly within this 1-h incubation were mostly fibroblasts (Rando and Blau 1994; Qu et al. 1998; Qu and Huard 2000a,Qu and Huard 2000b). The serial replating of the supernatant was repeated when 30–40% of the cells had adhered to each flask. After ∼5–6 serial platings, the culture was enriched with small, round cells (pp6; Qu et al. 1998; Qu and Huard 2000a,Qu and Huard 2000b).

Lee J.Y., Qu-Petersen Z., Cao B., Kimura S., Jankowski R., Cummins J., Usas A., Gates C., Robbins P., Wernig A, & Huard J. (2000). Clonal Isolation of Muscle-Derived Cells Capable of Enhancing Muscle Regeneration and Bone Healing. The Journal of Cell Biology, 150(5), 1085-1100.

Publication 2000

Cells Chick Embryo Collagen Culture Media Equus caballus Fibroblasts Mice, Inbred mdx Muscle Cells Penicillins Serum Streptomycin

Engineered Dystrophin Murine Model

Cited 7 times

The bases encoding amino acids 3402–3675 (corresponding to exons 71–78) were deleted from the full length murine dystrophin cDNA (sequence data available from EMBL/GenBank/DDBJ under accession No. M68859) by recombinant PCR, leaving the last three amino acids (exon 79) of the dystrophin protein unaltered. This dystrophin Δ71–78 cDNA was cloned into an expression vector containing bases −2139 to +239 of the human α-skeletal actin (HSA) promoter (Brennan and Hardeman 1993). A splice acceptor from the SV40 VP1 intron (isolated as a 400 bp HindIII/XbaI fragment from pSVL; Amersham Pharmacia Biotech) was inserted immediately 3′ of the HSA fragment, and the SV40 polyadenylation signal (isolated as a BamHI fragment from pCMVβ; MacGregor and Caskey 1989) was inserted 3′ of the dystrophin cDNA. The excised dystrophin Δ71–78 expression cassette was injected into wild-type C57Bl/10 × SJL/J F₂ hybrid embryos, and F₀ mice were screened by PCR. Five positive F₀'s were backcrossed onto the C57Bl/10mdx background, and most further studies focused on the line with the most uniform expression levels. Some studies used previously described transgenic mdx mice that express dystrophin constructs deleted approximately for exons 71–74 (Δ71–74) or exons 75–78 (Δ75–78), which remove amino acids 3402–3511 and 3528–3675, respectively (Rafael et al. 1996). Transgenic mdx line Dp71 expresses the Dp71 isoform of dystrophin in striated muscle (Cox et al. 1994).

Crawford G.E., Faulkner J.A., Crosbie R.H., Campbell K.P., Froehner S.C, & Chamberlain J.S. (2000). Assembly of the Dystrophin-Associated Protein Complex Does Not Require the Dystrophin Cooh-Terminal Domain. The Journal of Cell Biology, 150(6), 1399-1410.

Publication 2000

Actins Amino Acids Animals, Transgenic BP 400 Cloning Vectors DMD protein, human DNA, Complementary Embryo Exons Homo sapiens Hybrids Introns Mice, Inbred mdx Mice, Laboratory Mice, Transgenic Mus Muscle, Striated Polyadenylation Protein Isoforms Proteins Simian virus 40 Skeleton

Most recents protocols related to «Mice, Inbred mdx»

Gut Microbiota Alterations in DMD

In this study, we found that in mdx mice, a validated preclinical model of DMD, the disease is associated with a significant alteration in the gut microbiota composition compared with healthy controls. Along with this alteration, the plasma of mdx mice showed a reduction in the levels of gut microbiota‐related metabolites, the short‐chain fatty acids (SCFAs), and an elevation of those of endocannabinoids. Supplementation with the SCFA, sodium butyrate (NaB), rescued muscle strength and autophagy, and prevented inflammation associated with excessive endocannabinoid signaling at CB1 receptors to the same extent as deflazacort (DFZ), the standard palliative care for DMD. In C2C12 myoblasts stimulated with lipopolysaccharide, a pro‐inflammatory molecule derived from a malfunctioning gut microbiota, NaB exerted anti‐inflammatory effects, promoted autophagy, and prevented dysregulation of microRNAs that keep under negative control the CB1 receptor gene and did so in a manner depending on the activation of GPR109A and PPARγ receptors.

Kalkan H., Pagano E., Paris D., Panza E., Cuozzo M., Moriello C., Piscitelli F., Abolghasemi A., Gazzerro E., Silvestri C., Capasso R., Motta A., Russo R., Di Marzo V, & Iannotti F.A. (2023). Targeting gut dysbiosis against inflammation and impaired autophagy in Duchenne muscular dystrophy. EMBO Molecular Medicine, 15(3), e16225.

Publication 2023

Anti-Inflammatory Agents Autophagy deflazacort Endocannabinoids Fatty Acids, Volatile Gastrointestinal Microbiome Genes Inflammation Lipopolysaccharides Mice, Inbred mdx MicroRNAs Muscle Strength Myoblasts Palliative Care Plasma PPAR gamma Receptor, Cannabinoid, CB1 Sodium Butyrate

Gastrocnemius Protein Extraction and Analysis

Control and mdx mice were previously anesthetized with 75% CO₂/25% O₂ and then sacrificed by cervical dislocation. Gastrocnemius was rapidly dissected on ice and kept on dry ice until the whole procedure was completed. Muscle tissues were homogenized in 1x TNE buffer plus 1% (v/v) Triton X‐100 (Cat# T8787, Sigma‐Aldrich) protease Inhibitor (Cat# P8340, Sigma‐Aldrich) and phosphatase Inhibitor Cocktail 2 (Cat# P5726, Sigma‐Aldrich). Lysates were kept in an orbital shaker incubator at 220 rpm at 4°C for 30 min and then centrifuged for 15 min at 13,000 g at 4°C. The supernatants were transferred to tubes and quantified by DC Protein Assay (Cat# 5000116, Bio‐Rad, Milan, Italy). Subsequently, protein samples (60–80 μg of total protein) were heated at 70°C for 10 min in 1X LDS Sample Buffer (Cat# B0007, Life Technology) plus 1X sample reducing agent (Cat# B0009, Life Technology) and loaded on 4–12% Bis–Tris Protein Gels (Cat# NW04120, Life Technology) and then transferred the membrane using Trans‐Blot Turbo Mini 0.2 μm PVDF Transfer Packs (Cat# 1704156 Bio‐Rad). The primary antibodies used were (i) rabbit anti‐Akt Antibody (item n. 9272, Cell Signaling Technology USA); (ii) rabbit anti‐phospho Akt (Ser473) (D9E) XP^® (Cat# 4060, Cell Signaling Technology USA); (iii) rabbit anti‐LC3 antibody (Cat# 2775, Cell Signaling Technology USA); (iv) rabbit anti‐CB1 (Cat# Y409605, ABM Canada); (v) mouse anti‐CB2 (Cat# WH0001269MI, Merck); and (vi) an anti‐rabbit Cox2 (D5H5) XP^® (Cat# 12282, Cell Signaling ‐ USA). An anti‐GAPDH antibody (1D4) (Cat#. NB300‐221; Novus Biologicals) was used to check for equal protein loading. Reactive bands were detected by Clarity Western ECL Substrate (Cat# 1705061 Bio‐Rad). The intensity of bands was analyzed on a ChemiDoc station with Quantity‐one software (Bio‐Rad, Segrate, Italy).

Publication 2023

Antibodies Antibodies, Anti-Idiotypic Biological Assay Biological Factors Bistris Buffers Dry Ice GAPDH protein, human Gels Joint Dislocations Mice, Inbred mdx Mus Muscle, Gastrocnemius Muscle Tissue Neck Novus polyvinylidene fluoride Protease Inhibitors protein phosphatase inhibitor-2 Proteins PTGS2 protein, human Rabbits Reducing Agents Tissue, Membrane Triton X-100

Therapeutic Interventions in Muscular Dystrophy

The Animal Study Protocol (IACUC; 536/2018) was approved by the Italian Ministry of Health and Ethics Committee for the use of experimental animals being conformed to guidelines for the safe use and care of experimental animals following the Italian D.L. no. 116 of 27 January 1992 and associated guidelines in the European Communities Council (86/609/ECC and 2010/63/UE). In this study, 5‐week‐old control (C57BL/10ScSnJ) and dystrophic (C57BL/10ScSn‐DMDmdx/J) mice weighing approximately 20–25 g were purchased from Charles River Laboratories (Milan IT). All mice were housed in an individually ventilated cage system with a 12‐h light–dark cycle and received standard mouse chow (Harlan Teklad) and water ab libitum. Animals belonging to each cage were randomly assigned to the different experimental groups. Each experimental group included at least five mice. The experimenter(s) performing the treatments and locomotor testing was blind to the genotype and treatment. Control or mdx mice were treated orally for 3 weeks with (i) vehicle (dimethyl sulfoxide – DMSO Cat# 276855 Sigma‐Aldrich), (ii) deflazacort (DFZ) 1.2 mg/kg/day (Cat# SML0123 Sigma‐Aldrich), (iii) sodium butyrate (NaB) 100 mg/kg/day (Cat# 303410, Sigma‐Aldrich); (iii) ACEA 2.5 mg/Kg (Cat# A9719 Sigma‐Aldrich), or (iv) rimonabant 0.5 mg/Kg (Cat# 9000484, Cayman) were intraperitoneally (IP) injected three times a week for 2 weeks (Iannotti et al, 2018 (link)).

Publication 2023

Animals Animals, Laboratory Caimans deflazacort Ethics Committees Genotype Institutional Animal Care and Use Committees Mice, House Mice, Inbred mdx Rimonabant Rivers Sodium Butyrate Sulfoxide, Dimethyl Visually Impaired Persons

Generation of Fiona/dko Dystrophic Mice

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Publication 2023

5' Untranslated Regions Alleles DNA, Complementary Introns Mice, Inbred mdx Mice, Laboratory Oligonucleotide Primers Skeletal Muscles Transgenes UTRN protein, human Utrophin

Dystrophin-Deficient Mdx Mice for Dilated Cardiomyopathy

All animal experiments were approved by the animal care and use committee of the University of Missouri. All mice were maintained in a specific pathogen‐free animal care facility on a 12‐hour light (25 lux):12‐hour dark cycle with access to food and water ad libitum. Dystrophin‐deficient mdx mice (C57BL/10ScSn‐Dmd^mdx/J, stock number 001801) and normal control BL10 (wild‐type [WT]; C57BL/10ScSnJ, stock number 000476) mice were originally purchased from The Jackson Laboratory (Bar Harbor, ME). Experimental mice were generated in‐house in a barrier facility using breeders purchased from The Jackson Laboratory. Female mice were used in the current study for the following reasons. First, although DMD is an X‐linked disease, females can be afflicted if the mutation occurs in both alleles. Importantly, female patients display a characteristic DMD phenotype.²⁶ Second, while male mdx mice show more severe skeletal muscle disease, female mdx mice show more severe heart disease.²⁷, ²⁸ The classic presentation of DMD cardiac disease is dilated cardiomyopathy. This is observed in female, but not male, mdx mice.²⁷, ²⁹ The sample size shown in the figures refers to the number of mice used in the assay.

Morales E.D., Yue Y., Watkins T.B., Han J., Pan X., Gibson A.M., Hu B., Brito‐Estrada O., Yao G., Makarewich C.A., Babu G.J, & Duan D. (2023). Dwarf Open Reading Frame (DWORF) Gene Therapy Ameliorated Duchenne Muscular Dystrophy Cardiomyopathy in Aged mdx Mice. Journal of the American Heart Association: Cardiovascular and Cerebrovascular Disease, 12(3), e027480.

Publication 2023

Alleles Animals Biological Assay Cardiomyopathy, Dilated Females Food Heart Diseases Light Males Mice, House Mice, Inbred mdx Mutation Myopathy Patients Phenotype Specific Pathogen Free

Top products related to «Mice, Inbred mdx»

C57bl 10scsn dmdmdx j by Jackson ImmunoResearch

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C57BL/10ScSn-Dmdmdx/J is a mouse strain that carries a spontaneous mutation in the dystrophin gene, resulting in a model for Duchenne muscular dystrophy. This strain is commonly used in research related to muscular dystrophy and related disorders.

Mdx mice by Jackson ImmunoResearch

Sourced in United States

Mdx mice are a strain of genetically modified mice commonly used in research. They carry a mutation in the dystrophin gene, which is responsible for the production of the dystrophin protein. This mutation leads to a condition similar to Duchenne muscular dystrophy in humans. Mdx mice are a widely used model for studying the pathology and potential treatments for this genetic disorder.

C57bl 10scsnj by Jackson ImmunoResearch

Sourced in United States, Montenegro

The C57BL/10ScSnJ is a mouse strain commonly used in biomedical research. It serves as a standard control line for immunological and genetic studies.

C57bl 10scsn dmdmdx j mdx mice by Jackson ImmunoResearch

Sourced in United States, Montenegro

C57BL/10ScSn-Dmdmdx/J (mdx) mice are a strain of laboratory mice that carry a mutation in the dystrophin gene, which results in a lack of functional dystrophin protein. This mouse model is widely used in research to study Duchenne muscular dystrophy, a genetic disorder characterized by progressive muscle degeneration and weakness.

C57bl 6j by Jackson ImmunoResearch

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C57BL/6J is a mouse strain commonly used in biomedical research. It is a common inbred mouse strain that has been extensively characterized.

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C57BL/10ScSn is a mouse strain that serves as a source of tissues and cells for laboratory research. It is a sub-strain of the C57BL/10 mouse line.

C57bl 10scsn dmdmdx j mice by Jackson ImmunoResearch

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C57BL/10ScSn-Dmdmdx/J mice are a laboratory mouse strain that harbors a mutation in the dystrophin gene, resulting in a model for Duchenne muscular dystrophy. These mice exhibit progressive muscle weakness and degeneration, similar to the human condition.

C57bl 10 by Jackson ImmunoResearch

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C57BL/10 is a mouse model commonly used in immunological research. It is a subline of the C57BL/10 strain, which is known for its genetic stability and suitability for various experimental applications. The core function of this mouse model is to serve as a research tool for investigating immune system responses and disease processes.

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More about "Mice, Inbred mdx"

Duchenne muscular dystrophy (DMD) is a debilitating genetic disorder characterized by progressive muscle degeneration.
Inbred mdx mice, also known as C57BL/10ScSn-Dmdmdx/J mice, are a widely used animal model for studying this condition.
These mice harbor a mutation in the dystrophin gene, leading to the absence of functional dystrophin protein, similar to the human disease.
Researchers utilize inbred mdx mice to investigate the underlying pathogenesis of DMD, evaluate potential therapies, and develop new treatment strategies.
The C57BL/10ScSnJ and C57BL/6J mouse strains are often used as control groups in mdx mouse studies.
PubCompare.ai's AI-driven platform can help optimize your mdx mouse research by identifying the most reproducible and accurate protocols from published literature, preprints, and patents.
With intelligent comparisons, you can easily locate the best products and procedures, such as the Grip strength meter and TRIzol reagent, to advance your studies and accelerate the development of new therapies for Duchenne muscular dystrophy.
By leveraging the power of data-driven decision making, you can experience the benefits of PubCompare.ai's platform and make informed choices to improve the efficiency and accuracy of your mdx mouse research, ultimately contributing to the progress in understanding and treating this debilitating condition.