IHC 1:150-1:500, IF 1: 50-1:200, ELISA 1:5000-1:240000
Restrictions
For Research Use only
Format
Liquid
Concentration
2.6 mg/mL
Buffer
PBS with 0.05 % Sodium azide and 40 % Glycerol, pH 7.4
Preservative
Sodium azide
Precaution of Use
This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
Storage
-20 °C
Storage Comment
Store at -20°C. Avoid freeze / thaw cycles.
Target
VMA21
(Vacuolar H+-ATPase Homolog (VMA21))
Alternative Name
VMA21
Background
This gene encodes a chaperone for assembly of lysosomal vacuolar ATPase. Required for the assembly of the V0 complex of the vacuolar ATPase (V-ATPase) in the endoplasmic reticulum. Associates with the V0 complex of the vacuolar ATPase (V-ATPase). 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.