VMA21 Antibody (PACO13179)
- SKU:
- PACO13179
- Product Type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Applications:
- ELISA
- Applications:
- WB
- Antibody Type:
- Polyclonal Antibody
- Conjugation:
- Unconjugated
Description
| Antibody Name: | VMA21 Antibody (PACO13179) |
| Antibody SKU: | PACO13179 |
| Size: | 50ul |
| Host Species: | Rabbit |
| Tested Applications: | ELISA, WB |
| Recommended Dilutions: | |
| Species Reactivity: | Human, Mouse, Rat |
| Immunogen: | Human VMA21 |
| Form: | Liquid |
| Storage Buffer: | PBS with 0.02% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles. |
| Purification Method: | Antigen Affinity purified |
| Clonality: | Polyclonal |
| Isotype: | IgG |
| Conjugate: | Non-conjugated |
| Synonyms: | VMA21 vacuolar H+-ATPase homolog (S. cerevisiae);VMA21;MGC125514;MGC125516;MGC131652 ; |
| UniProt Protein Function: | 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-passChromosomal Location of Human Ortholog: Xq28Cellular Component: endoplasmic reticulum membrane; ER-Golgi intermediate compartment membrane; COPII vesicle coat; lysosome; integral to membraneDisease: Myopathy, X-linked, With Excessive Autophagy |
| UniProt Protein Details: | |
| NCBI Summary: | This gene encodes a chaperone for assembly of lysosomal vacuolar ATPase.[provided by RefSeq, Jul 2012] |
| UniProt Code: | Q3ZAQ7 |
| NCBI GenInfo Identifier: | 63025214 |
| NCBI Gene ID: | 203547 |
| NCBI Accession: | NP_001017980 |
| UniProt Secondary Accession: | Q3ZAQ7 |
| UniProt Related Accession: | Q3ZAQ7 |
| Molecular Weight: | 11kDa |
| NCBI Full Name: | vacuolar ATPase assembly integral membrane protein VMA21 isoform 1 |
| NCBI Synonym Full Names: | vacuolar ATPase assembly factor VMA21 |
| NCBI Official Symbol: | VMA21 |
| NCBI Official Synonym Symbols: | MEAX; XMEA |
| NCBI Protein Information: | vacuolar ATPase assembly integral membrane protein VMA21 |
| UniProt Protein Name: | Vacuolar ATPase assembly integral membrane protein VMA21 |
| UniProt Synonym Protein Names: | Myopathy with excessive autophagy protein |
| Protein Family: | Vacuolar ATPase assembly integral membrane protein |
| UniProt Gene Name: | VMA21 |
| UniProt Entry Name: | VMA21_HUMAN |
Related Products
SIM1 Antibody
Overview Product Name:SIM1 AntibodyProduct Code:PACO64949Size:50µLTarget Names:SIM1Species Reactivity:HumanHost
eutC Antibody
Overview Product Name:eutC AntibodyProduct Code:PACO64709Size:50µLTarget Names:eutCSpecies Reactivity:Escherichia coli
EPHA10 Antibody
Overview Product Name:EPHA10 AntibodyProduct Code:PACO64693Size:50µLTarget Names:EPHA10Species Reactivity:HumanH
otsB Antibody
Overview Product Name:otsB AntibodyProduct Code:PACO64885Size:50µLTarget Names:otsBSpecies Reactivity:Escherichia coli
PCDHA8 Antibody
Overview Product Name:PCDHA8 AntibodyProduct Code:PACO64893Size:50µLTarget Names:PCDHA8Species Reactivity:HumanH