Invitrogen

Dystrophin Polyclonal Antibody

Tech Support

Datasheet

Protocols

Questions & Answers

Tech Support

Product Image Gallery

Dystrophin Antibody in Immunohistochemistry (IHC) — IHC analysis of horse muscle tissues using Product # PA1-37587. Horse muscle sections (8 µm) were blocked with 5% horse serum in 1X PBS and stained with PA1-37587 (1:50) for 1 hour followed by a fluorophore-conjugated anti-rabbit IgG secondary antibody (red). Tissues were also stained for Myosin Heavy chain type IIA (green). Data courtesy of the Innovators Program.

Dystrophin Antibody in Immunocytochemistry (ICC/IF) — Published figure using Dystrophin polyclonal antibody (Product # PA5-32388) in Immunocytochemistry

Dystrophin Antibody in Immunohistochemistry (Frozen) (IHC (F)) — Muscle fiber type composition is a major determinant in fatty acid-supported mitochondrial respiration. (A), (B), (C) Representative imaging of muscle fiber typing via Myosin Heavy Chain (MyHC) immunohistochemistry in predominately glycolytic (EDL), mixed (TA), and oxidative (Soleus) muscles. Magnification 20X. Scale bar 200 mum. (D) Mitochondrial respiration ( JO 2 ) across a continuum of ATP free energy (DeltaG ATP ) and (E) respiratory conductance in predominantly glycolytic, mixed, and oxidative muscles energized with palmitoylcarnitine/malate (PC/M). (F) Heatmap of abundance of proteins involved in mitochondrial fatty acid oxidation in glycolytic (EDL) and oxidative (SOL) muscles. (G) Relative abundance of long-chain acylcarnitines (LCACs) in mixed TA muscle. (H) Digital reconstitution of mass spectroscopy-based lipid scanning (nano-DESI) of palmitoleyl-carnitine (C16:1) in mixed TA muscle and (I) immunostaining for myosin heavy chain type 2A (green) and dystrophin (white) on consecutive muscle section. For (D), (E), and (G), data is presented as Mean +- SEM; n = 3-6 and *P <= 0.05 by 2-way ANOVA. For (F), data is presented as log2 abundance; n = 6 and *P <= 0.05 of adjusted p-value calculated by Benjamini Hochberg FDR correction. Figure 1

Dystrophin Antibody in Immunohistochemistry, Immunohistochemistry (PFA fixed) (IHC, IHC (PFA)) — Fig. 3. Reduced mass and myofiber area in Sgcg 521DeltaT mice. (A) Body mass was not significantly different among genotypes. (B) Gluteus/hamstring (GH) muscle mass normalized to tibia length was reduced in 521DeltaT mice compared with controls. (C) The total mass from muscle groups normalized to tibia length was significantly reduced in 521DeltaT mice compared with WT controls (quadriceps, gluteus/hamstring, triceps, diaphragm, heart, tibialis anterior muscle groups combined). (D) Average myofiber cross-sectional area (CSA) was significantly smaller in 521DeltaT mice than in WT controls. (E) Percentage of myofibers with >1 internal nuclei was increased in 521DeltaT muscle, with 4-month-old 521DeltaT myofibers having the most internal nuclei. (F) Representative images of 521DeltaT and WT muscle. Anti-dystrophin (green) outlined myofibers. Hoechst (blue) demonstrated nuclei. Data are mean+-s.e.m. * P <0.05 (one-way ANOVA with Tukey; n >=5 mice per group). Scale bars: 50 um.

Dystrophin Antibody in Western Blot (WB) — Fig. 2. Characterization of DMD and CRISPR-corrected PSC-derived MPCs and myotubes. ( A ) Timeline of the myogenic specification from human ePSCs to MPCs and myotubes with markers for each specific stage. ( B ) Secondary differentiation of MPCs into myotubes. Experiments are analyzed at 0 hours (MPCs in skeletal muscle cell growth medium) and at 24, 48, and 120 hours after switching to secondary differentiation medium. ( C ) Representative immunocytochemistry images of PAX7, MYOD1, dystrophin, MYH (MF20), and titin in DMD-R3381X and CORR-R3381X myogenic cultures at 120 hours of secondary differentiation. Enlarged images of titin demonstrate sarcomere striation. Scale bars, 100 mum. ( D ) Immunoblotting confirms that full-length dystrophin (427 kDa) is restored in CORR-R3381X myogenic cultures. ( E ) Quantification of PAX7-positive, MYOD1-positive, and MF20-positive cells and fusion index during secondary differentiation at 0, 24, 48, and 120 hours in DMD-R3381X and CORR-R3381X cultures. Values are means +- SD. n = 6. Two-way analysis of variance (ANOVA) and Sidak's multiple comparisons test, ** P < 0.01, *** P < 0.001, and **** P < 0.0001. ( F ) Heatmap of normalized gene expression levels as log 2 transformed RPKM (reads per kilobase per million mapped reads) of myogenic markers in DMD-R3381X and CORR-R3381X cells at 0, 24, and 120 hours of secondary differentiation. ( G ) Unsupervised cluster dendrogram of DMD-R3381X and CORR-R3381X transcriptomes at 0, 24, and 120 hours of

Product Details

PA5-32388

Applications

Tested Dilution

Publications

Western Blot (WB)

View 2 publications 2 publications

Immunohistochemistry (IHC)

View 6 publications 6 publications

Immunohistochemistry (Paraffin) (IHC (P))

1:100

Immunohistochemistry (PFA fixed) (IHC (PFA))

View 1 publication 1 publication

Immunohistochemistry (Frozen) (IHC (F))

1:400

View 3 publications 3 publications

Immunocytochemistry (ICC/IF)

View 3 publications 3 publications

Product Specifications

Species Reactivity

Human, Mouse

Published species

Bovine, Human, Mouse, Rat

Host/Isotype

Rabbit / IgG

Class

Polyclonal

Type

Antibody

Immunogen

Synthetic peptide derived from C-terminus of human dystrophin.

View immunogen

Conjugate

Unconjugated

Form

Liquid

Concentration

Conc. not determined

Purification

Antigen affinity chromatography

Storage buffer

PBS, pH 7.6, with 1% BSA

Contains

0.1% sodium azide

Storage conditions

-20° C, Avoid Freeze/Thaw Cycles

RRID

AB_2549858

Product Specific Information

This antibody is predicted to react with bovine, chicken, canine and porcine based on sequence homology.

Heat-mediated antigen retrieval is recommended prior to staining, using a 10mM citrate buffer, pH 6.0, for 10 minutes followed by cooling at room temperature for 20 min. Following antigen retrieval, incubate samples with primary antibody for 10 min at room temperature. A suggested positive control is skeletal muscle tissue.

Target Information

Dystrophin is the 427kDa protein product of the DMB/BMD gene located on the X chromosome at position Xp21. Western blotting and immunohistochemistry are the two established methods for the detection of abnormalities of dystrophin expression in muscle biopsies. Dystrophin abnormalities are thought to occur in 100% of patients with DMD/BMD, although genetic abnormalities may only be detected in up to 65% of cases.

For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.

Product References

The adhesion G-protein-coupled receptor Gpr116 is essential to maintain the skeletal muscle stem cell pool.

Cell reports

Sénéchal C,Fujita R,Jamet S,Maiga A,Dort J,Orfi Z,Dumont NA,Bouvier M,Crist C

PA1-37587 was used in Western Blotting to propose a model by which GPR116 maintains the MuSC pool via nuclear functions of β-arrestin1.

Tue Nov 15 00:00:00 EST 2022

Skeletal muscle undergoes fiber type metabolic switch without myosin heavy chain switch in response to defective fatty acid oxidation.

Molecular metabolism

Pereyra AS,Lin CT,Sanchez DM,Laskin J,Spangenburg EE,Neufer PD,Fisher-Wellman K,Ellis JM

Published figure using Dystrophin polyclonal antibody (Product # PA5-32388) in Immunohistochemistry (Frozen)

Sun May 01 00:00:00 EDT 2022

Intermittent glucocorticoid treatment enhances skeletal muscle performance through sexually dimorphic mechanisms.

The Journal of clinical investigation

Salamone IM,Quattrocelli M,Barefield DY,Page PG,Tahtah I,Hadhazy M,Tomar G,McNally EM

PA1-37587 was used in Immunohistochemistry to investigate the impact of once-weekly glucocorticoid exposure on skeletal muscle performance comparing male and female mice.

Tue Mar 15 00:00:00 EDT 2022

Optogenetic modeling of human neuromuscular circuits in Duchenne muscular dystrophy with CRISPR and pharmacological corrections.

Science advances

Paredes-Redondo A,Harley P,Maniati E,Ryan D,Louzada S,Meng J,Kowala A,Fu B,Yang F,Liu P,Marino S,Pourquié O,Muntoni F,Wang J,Lieberam I,Lin YY

PA5-32388 was used in Western Blot to provide a new human microphysiological model for investigating neuromuscular junction defects in duchenne muscular dystrophy.

Fri Sep 10 00:00:00 EDT 2021

Optogenetic modeling of human neuromuscular circuits in Duchenne muscular dystrophy with CRISPR and pharmacological corrections.

Science advances

Paredes-Redondo A,Harley P,Maniati E,Ryan D,Louzada S,Meng J,Kowala A,Fu B,Yang F,Liu P,Marino S,Pourquié O,Muntoni F,Wang J,Lieberam I,Lin YY

PA5-32388 was used in Western Blot to provide a new human microphysiological model for investigating neuromuscular junction defects in duchenne muscular dystrophy.

Fri Sep 10 00:00:00 EDT 2021

LAT1 Protein Content Increases Following 12 Weeks of Resistance Exercise Training in Human Skeletal Muscle.

Frontiers in nutrition

Roberson PA,Mobley CB,Romero MA,Haun CT,Osburn SC,Mumford PW,Vann CG,Greer RA,Ferrando AA,Roberts MD

Published figure using Dystrophin polyclonal antibody (Product # PA5-32388) in Immunohistochemistry

Wed Feb 03 00:00:00 EST 2021

Synergist ablation-induced hypertrophy occurs more rapidly in the plantaris than soleus muscle in rats due to different molecular mechanisms.

American journal of physiology. Regulatory, integrative and comparative physiology

Roberts MD,Mobley CB,Vann CG,Haun CT,Schoenfeld BJ,Young KC,Kavazis AN

PA5-32388 was used in Immunohistochemistry to examine molecular mechanisms that are altered during rapid soleus (type I fiber-dominant) and plantaris (type II fiber-dominant) hypertrophy in rats.

Sat Feb 01 00:00:00 EST 2020

A gene-edited mouse model of limb-girdle muscular dystrophy 2C for testing exon skipping.

Disease models & mechanisms

Demonbreun AR,Wyatt EJ,Fallon KS,Oosterbaan CC,Page PG,Hadhazy M,Quattrocelli M,Barefield DY,McNally EM

Published figure using Dystrophin polyclonal antibody (Product # PA5-32388) in Immunohistochemistry

Mon Nov 04 00:00:00 EST 2019

A gene-edited mouse model of limb-girdle muscular dystrophy 2C for testing exon skipping.

Disease models & mechanisms

Demonbreun AR,Wyatt EJ,Fallon KS,Oosterbaan CC,Page PG,Hadhazy M,Quattrocelli M,Barefield DY,McNally EM

Published figure using Dystrophin polyclonal antibody (Product # PA5-32388) in Immunohistochemistry

Mon Nov 04 00:00:00 EST 2019

CRISPR/Cas9 Technology in Restoring Dystrophin Expression in iPSC-Derived Muscle Progenitors.

Journal of visualized experiments : JoVE

Jin Y,Shen Y,Su X,Weintraub N,Tang Y

PA5-32388 was used in Immunocytochemistry to show the feasibility of using CRISPR/Cas9 deletion strategy to restore dystrophin expression in iPSC-derived MPC, which has significant potential for developing future therapies for the treatment of DMD.

Sat Sep 14 00:00:00 EDT 2019

CRISPR/Cas9 Technology in Restoring Dystrophin Expression in iPSC-Derived Muscle Progenitors.

Journal of visualized experiments : JoVE

Jin Y,Shen Y,Su X,Weintraub N,Tang Y

Sat Sep 14 00:00:00 EDT 2019

Myoblots: dystrophin quantification by in-cell western assay for a streamlined development of Duchenne muscular dystrophy (DMD) treatments.

Neuropathology and applied neurobiology

Ruiz-Del-Yerro E,Garcia-Jimenez I,Mamchaoui K,Arechavala-Gomeza V

PA5-32388 was used in Immunocytochemistry to quantify dystrophin and other muscle-associated proteins in muscle cell cultures to distinguish accurately between the different sets of patients based on their dystrophin expression and detect dystrophin restoration after treatment.

Wed Aug 01 00:00:00 EDT 2018

Effect of Ibuprofen on Skeletal Muscle of Dysferlin-Null Mice.

The Journal of pharmacology and experimental therapeutics

Collier AF,Gumerson J,Lehtimäki K,Puoliväli J,Jones JW,Kane MA,Manne S,O'Neill A,Windish HP,Ahtoniemi T,Williams BA,Albrecht DE,Bloch RJ

PA5-32388 was used in Immunohistochemistry-immunofluorescence to indicate that ibuprofen can have deleterious effects on dysferlin-null muscle and suggest that its use at pharmacological doses should be avoided by individuals with dysferlinopathies.

Thu Mar 01 00:00:00 EST 2018

Direct reprogramming of urine-derived cells with inducible MyoD for modeling human muscle disease.

Skeletal muscle

Kim EY,Page P,Dellefave-Castillo LM,McNally EM,Wyatt EJ

PA1-37587 was used in immunocytochemistry discuss different cell sources from which to generate muscle cells

Mon Oct 23 00:00:00 EDT 2017

Phosphorylation of eIF2α Is a Translational Control Mechanism Regulating Muscle Stem Cell Quiescence and Self-Renewal.

Cell stem cell

Zismanov V,Chichkov V,Colangelo V,Jamet S,Wang S,Syme A,Koromilas AE,Crist C

PA1-37587 was used in immunohistochemistry study the role of eIF2alpha in skeletal muscle stem cells

Thu Jan 07 00:00:00 EST 2016

Effect of extended postmortem aging and steak location on myofibrillar protein degradation and Warner-Bratzler shear force of beef M. semitendinosus steaks.

Journal of animal science

Phelps KJ,Drouillard JS,Silva MB,Miranda LD,Ebarb SM,Van Bibber-Krueger CL,O'Quinn TG,Gonzalez JM

PA1-37587 was used in immunohistochemistry - frozen section to study myofibrillar protein degradation and Warner-Bratzler shear force of beef M. semitendinosus steaks and the effect of extended postmortem aging and steak location

Fri Jan 01 00:00:00 EST 2016

Realimentation of nutrient restricted pregnant beef cows supports compensatory fetal muscle growth.

Journal of animal science

Gonzalez JM,Camacho LE,Ebarb SM,Swanson KC,Vonnahme KA,Stelzleni AM,Johnson SE

PA1-37587 was used in immunohistochemistry - frozen section to study the effect of nutrient restriction at specific gestational timeframes on fetal skeletal muscle growth

Tue Oct 01 00:00:00 EDT 2013

Bioinformatics

Protein Aliases: Duchenne muscular dystrophy (DMD); Dystrophin; dystrophin Dp40; dystrophin Dp71a; dystrophin Dp71ab; dystrophin Dp71b; GS1-19O24.1; Muscular dystrophy Duchenne and Becker types; OTTHUMP00000215592; X-linked muscular dystrophy

Gene Aliases: BMD; CMD3B; DMD; Dp427; Dp71; DXS142; DXS164; DXS206; DXS230; DXS239; DXS268; DXS269; DXS270; DXS272; DXSmh7; DXSmh9; dys; mdx; MRX85; pke

UniProt ID: (Human) E7EQS5, (Mouse) P11531

Entrez Gene ID: (Human) 1756, (Mouse) 13405

Molecular Function: ubiquitin-protein ligase