GYG1 monoclonal antibody (M07), clone 3B5
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More Files
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Specification
Product Description
Mouse monoclonal antibody raised against a partial recombinant GYG1.
Immunogen
GYG1 (NP_004121, 1 a.a. ~ 73 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.
Sequence
MTDQAFVTLTTNDAYAKGALVLGSSLKQHRTTRRLVVLATPQVSDSMRKVLETVFDEVIMVDVLDSGDSAHLT
Host
Mouse
Reactivity
Human, Rat
Interspecies Antigen Sequence
Mouse (95); Rat (97)
Isotype
IgG2a Kappa
Quality Control Testing
Antibody Reactive Against Recombinant Protein.
Western Blot detection against Immunogen (33.77 KDa) .
Storage Buffer
In 1x PBS, pH 7.4
Storage Instruction
Store at -20°C or lower. Aliquot to avoid repeated freezing and thawing.
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Applications
Western Blot (Cell lysate)
GYG1 monoclonal antibody (M07), clone 3B5 Western Blot analysis of GYG1 expression in HepG2 ( Cat # L019V1 ).Western Blot (Cell lysate)
GYG1 monoclonal antibody (M07), clone 3B5. Western Blot analysis of GYG1 expression in PC-12 ( Cat # L012V1 ).Western Blot (Transfected lysate)
Western Blot analysis of GYG1 expression in transfected 293T cell line by GYG1 monoclonal antibody (M07), clone 3B5.
Lane 1: GYG1 transfected lysate(37.5 KDa).
Lane 2: Non-transfected lysate.
Western Blot (Recombinant protein)
Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections)
Immunoperoxidase of monoclonal antibody to GYG1 on formalin-fixed paraffin-embedded human testis. [antibody concentration 3 ug/ml]Sandwich ELISA (Recombinant protein)
Detection limit for recombinant GST tagged GYG1 is approximately 0.03ng/ml as a capture antibody.ELISA
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Gene Info — GYG1
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Interactome
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Publication Reference
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A FLCN-TFE3 Feedback Loop Prevents Excessive Glycogenesis and Phagocyte Activation by Regulating Lysosome Activity.
Endoh M, Baba M, Endoh T, Hirayama A, Nakamura-Ishizu A, Umemoto T, Hashimoto M, Nagashima K, Soga T, Lang M, Schmidt LS, Linehan WM, Suda T.
Cell Reports 2020 Feb; 30(6):1823.
Application:WB-Ce, Mouse, Mouse spleen cells.
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Functional characterization of GYG1 variants in two patients with myopathy and glycogenin-1 deficiency.
Carola Hedberg-Oldfors, Willem De Ridder, Ognian Kalev, Klaus Böck, Kittichate Visuttijai, Georg Caravias, Ana Töpf, Volker Straub, Jonathan Baets, Anders Oldfors.
Neuromuscular Disorders : NMD 2019 Dec; 29(12):951.
Application:WB-Ti, Human, Human skeletal muscle.
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Glycogenin is dispensable for glycogen synthesis in human muscle and glycogenin deficiency causes polyglucosan storage.
Visuttijai K, Carola HO, Thomsen C, Glamuzina E, Kornblum C, Tasca G, Hernandez-Lain A, Sandstedt J, Dellgren G, Roach P, Oldfors A.
The Journal of Clinical Endocrinology and Metabolism 2019 Oct; dgz075.
Application:IHC-Fr, IHC-P, WB-Ti, Human, Livers, Hearts, Skeletal muscles.
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Clinical heterogeneity and phenotype/genotype findings in 5 families with GYG1 deficiency.
Ben Yaou R, Hubert A, Nelson I, Dahlqvist JR, Gaist D, Streichenberger N, Beuvin M, Krahn M, Petiot P, Parisot F, Michel F, Malfatti E, Romero N, Carlier RY, Eymard B, Labrune P, Duno M, Krag T, Cerino M, Bartoli M, Bonne G, Vissing J, Laforet P, Petit FM.
Neurology. Genetics 2017 Dec; 3(6):e208.
Application:WB-Ce, Human, Myoblast cell culture from patient A-1 and frozen muscle tissue from patients.
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Cardiomyopathy as presenting sign of glycogenin-1 deficiency-report of three cases and review of the literature.
Hedberg-Oldfors C, Glamuzina E, Ruygrok P, Anderson LJ, Elliott P, Watkinson O, Occleshaw C, Abernathy M, Turner C, Kingston N, Murphy E, Oldfors A.
Journal of Inherited Metabolic Disease 2017 Jan; 40(1):139.
Application:WB, Human, Cardiac muscle biopsy specimen.
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Start codon mutation of GYG1 causing late-onset polyglucosan body myopathy with nemaline rods.
Tasca G, Fattori F, Monforte M, Hedberg-Oldfors C, Sabatelli M, Udd B, Boldrini R, Bertini E, Ricci E, Oldfors A.
Journal of Neurology 2016 Oct; 263(10):2133.
Application:WB-Ti, Human, Muscle.
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A new muscle glycogen storage disease associated with Glycogenin-1 deficiency.
Malfatti E, Nilsson J, Hedberg-Oldfors C, Hernandez-Lain A, Michel F, Dominguez-Gonzalez C, Viennet G, Akman HO, Kornblum C, Van den Bergh P, Romero NB, Engel AG, DiMauro S, Oldfors A.
Annals of Neurology 2014 Dec; 76(6):891.
Application:WB-Ti, Human, Skeletal muscle.
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LC-MS/MS characterization of combined glycogenin-1 and glycogenin-2 enzymatic activities reveals their self-glucosylation preferences.
Nilsson J, Halim A, Larsson E, Moslemi AR, Oldfors A, Larson G, Nilsson J.
Biochimica et Biophysica Acta 2014 Feb; 1844(2):398.
Application:WB-Ti, Human, Liver, Skeletal muscle, Heart, Adipose.
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Dysregulation of multiple facets of glycogen metabolism in a murine model of pompe disease.
Taylor KM, Meyers E, Phipps M, Kishnani PS, Cheng SH, Scheule RK, Moreland RJ.
PLoS One 2013 Feb; 8(2):e56181.
Application:WB, Mouse, Mouse muscle.
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Molecular pathogenesis of a new glycogenosis caused by a glycogenin-1 mutation.
Nilsson J, Halim A, Moslemi AR, Pedersen A, Nilsson J, Larson G, Oldfors A.
Biochimica et Biophysica Acta 2011 Dec; 1822(4):493.
Application:WB, Human, UDP-glucose.
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A FLCN-TFE3 Feedback Loop Prevents Excessive Glycogenesis and Phagocyte Activation by Regulating Lysosome Activity.
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