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品种反应性 主要应用 宿主 格式 抗体类型 Chp, H, R, M, Rhesus Macaque IHC, WB, ICC Rb Affinity Purified Polyclonal Antibody 描述 产品目录编号 06-1008 描述 Anti-VMA21 Antibody Alternate Names - mNET4
- VMA21 vacuolar H+-ATPase homolog (S. cerevisiae)
- Myopathy with excessive autophagy protein
- vacuolar ATPase assembly integral membrane protein VMA21
- myopathy with excessive autophagy1
背景信息 VMA21, also known as NET4, is a necessary assembly chaperone for the V-ATPase proton pump of the cellular membrane. Deficiency of VMA21 is thought to be associated with XMEA (X-linked myopathy with excessive autophagy) which causes progressive vacuolation and skeletal muscle atrophy. 产品信息 格式 Affinity Purified 控制 - Human fetal skeletal muscle tissue lysate.
演示 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide. 应用 应用 Detect VMA21 using this Anti-VMA21 Antibody validated for use in WB, IC, IH. 主要应用 - Immunohistochemistry
- Western Blotting
- Immunocytochemistry
应用说明 Immunohistochemistry Analysis: A representative lot was used by an independent laboratory in IH. (Wilkie, G.S., et al. (2011). Mol Cell Proteomics. 10(1)).
Immunocytochemistry Analysis: A representative lot was used by an independent laboratory in IC. (Wilkie, G.S., et al. (2011). Mol Cell Proteomics. 10(1).生物信息 免疫原品种 KLH-conjugated linear peptide corresponding to the N-terminus of human VMA21. 表位 N-terminus 浓缩 Please refer to the Certificate of Analysis for the lot-specific concentration. 宿主 Rabbit 特异性 This antibody recognizes the N-terminus of VMA21. 品种反应性 ChimpanzeeHumanRatMouseRhesus Macaque Species Reactivity Note Demonstrated to react with Human, Rat, and Mouse. Predicted to react with Rhesus Macaque, Orangutan, and Chimpanzee based on 100% sequence homology. 抗体类型 Polyclonal Antibody Entrez基因编号 - NP_001017980
Entrez基因汇总 Summary: This gene encodes a vacuolar ATPase assembly integral membrane protein. Mutations of this gene result in an X-linked vacuolar myopathy with excessive autophagy. 基因符号 - MEAX
- XMEA
纯化方法 Affinity purified UniProt编号 - Q3ZAQ7
UniProt汇总 FUNCTION: Required for the assembly of the V0 complex of the vacuolar ATPase (V-ATPase) in the endoplasmic reticulum.
SUBUNIT STRUCTURE: Associates with the V0 complex of the vacuolar ATPase (V-ATPase).
SUBCELLULAR LOCATION: Endoplasmic reticulum membrane; Multi-pass membrane protein. Endoplasmic reticulum-Golgi intermediate compartment membrane; Multi-pass membrane protein. Cytoplasmic vesicle › COPII-coated vesicle membrane; Multi-pass membrane protein.
INVOLVEMENT IN DISEASE: Defects in VMA21 are the cause of X-linked myopathy with excessive autophagy (MEAX) [MIM:310440]. MEAX is a childhood-onset disease characterized by progressive vacuolation and atrophy of skeletal muscle. It is inherited in recessive fashion, affecting boys and sparing carrier females. Onset is in childhood, and patients exhibit weakness of the proximal muscles of the lower extremities, progressing slowly to involve other skeletal muscle groups over time. Other organs including the heart and brain are clinically unaffected. Phenotype is due to an increase of lysosomal pH from 4.7 to 5.2, which reduces lysosomal degradative ability and blocks autophagy. This reduces cellular free amino acids, which upregulates the mTOR pathway and mTOR-dependent macroautophagy, resulting in proliferation of large and ineffective autolysosomes that engulf sections of cytoplasm, merge together, and vacuolate the cell.
SEQUENCE SIMILARITIES: Belongs to the VMA21 family.
CAUTION: Protein characterization data are from. Due to a number of errors in the figure panels, the article has been retracted but the authors stand by the validity of the main results and conclusions.产品使用声明 质量保证 Evaluated by Western Blot in human fetal skeletal muscle tissue lysate.
Western Blot Analysis: 1 µg/mL of this antibody detected VMA21 on 10 µg of human fetal skeletal muscle tissue lysate.使用声明 - Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
储存和货运信息 存储条件 Stable for 1 year at 2-8°C from date of receipt. 包装信息 数量 100 µg
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