VMA21, Polyclonal Antibody

Anti -VMA21, NT (Vacuolar ATPase Assembly Integral Membrane Protein VMA21, Myopathy with Excessive Autophagy Protein, MEAX, XMEA)

For Research Use Only. Not for use in diagnostic procedures.

Chromosome: X; NC_000023.10 (150565657..150577836). Location: Xq28

Polyclonal

Rabbit

Recognizes human VMA21. Species Crossreactivity

Affinity Purified
Purified by immunoaffinity chromatography.

Supplied as a liquid in 0.1M Tris-glycine, pH 7.4, 150mM sodium chloride, 0.05% sodium azide.

May be stored at 4 degree C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degree C. Aliquots are stable for at least 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Small volumes of anti-VMA21 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

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.

Antibodies; Abs to Enzymes, ATPase

Western Blot (WB), Immunohistochemistry (IHC), Immunocytochemistry (ICC)

Suitable for use in Western Blot, Immunohistochemistry and Immunocytochemistry.
Dilution: Western Blot: 1ug/ml

11,354 Da[Similar Products]

VMA21 vacuolar H+-ATPase homolog (S. cerevisiae)

vacuolar ATPase assembly integral membrane protein VMA21; myopathy with excessive autophagy protein

Vacuolar ATPase assembly integral membrane protein VMA21

MEAX; XMEA  [Similar Products]

VMA21_HUMAN

This gene encodes a chaperone for assembly of lysosomal vacuolar ATPase.[provided by RefSeq, Jul 2012]

VMA21: Required for the assembly of the V0 complex of the vacuolar ATPase (V-ATPase) in the endoplasmic reticulum. Defects in VMA21 are the cause of X-linked myopathy with excessive autophagy (MEAX). 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 up-regulates 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. Belongs to the VMA21 family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Membrane protein, integral; Membrane protein, multi-pass

Chromosomal Location of Human Ortholog: Xq28

Cellular Component: endoplasmic reticulum membrane; ER-Golgi intermediate compartment membrane; COPII vesicle coat; lysosome; integral to membrane

Disease: Myopathy, X-linked, With Excessive Autophagy

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