Hexosaminidase A Alpha (HEXa) Antibody

Product specifications

Primary Antibodies

Hexosaminidase A Alpha (HEXa)

Rabbit

Human

WB: 0.01-2 µg/ml, IHC: 5-20 µg/ml, IF/ICC: 5-20 µg/ml. Optimal dilutions/concentrations should be determined by the end user.

Polyclonal

Unconjugated

Purified by antigen-specific affinity chromatography, followed by Protein A affinity chromatography.

100 µl

200 µl

1 ml

Liquid

WB, IHC, IF/ICC

0.01 M PBS, pH 7.4, containing 0.05% Proclin-300, 50% glycerol.

Shipped within 5-7 working days.

Aliquot and store at -20°C. Avoid repeated freeze/thaw cycles.

No

P06865

N-acetyl-beta-glucosaminidase subunit alpha,TSD

Antibody anti-HEXA

RUO

Descripción

HEXA is the alpha subunit of the beta-hexosaminidase enzyme, a lysosomal hydrolase that catalyzes the breakdown of GM2 gangliosides into GM3 in the process of glycosphingolipid degradation The enzyme functions as a heterodimer composed of alpha and beta subunits, encoded by the HEXA and HEXB genes respectively Mutations in HEXA lead to Tay-Sachs disease, an autosomal recessive lysosomal storage disorder characterized by the accumulation of GM2 gangliosides in neuronal cells, resulting in neurodegeneration, progressive motor weakness, and developmental delay HEXA is highly active in neuronal tissues where glycosphingolipid metabolism is critical for normal brain function The absence or dysfunction of HEXA causes a loss of enzymatic activity leading to cellular toxicity and apoptosis Current therapeutic approaches include enzyme replacement therapy, substrate reduction therapy, and gene therapy to restore HEXA activity and slow disease progression

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