HEXA rabbit pAb

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Background

This gene encodes a member of the glycosyl hydrolase 20 family of proteins. The encoded preproprotein is proteolytically processed to generate the alpha subunit of the lysosomal enzyme beta-hexosaminidase. This enzyme, together with the cofactor GM2 activator protein, catalyzes the degradation of the ganglioside GM2, and other molecules containing terminal N-acetyl hexosamines. Mutations in this gene lead to an accumulation of GM2 ganglioside in neurons, the underlying cause of neurodegenerative disorders termed the GM2 gangliosidoses, including Tay-Sachs disease (GM2-gangliosidosis type I) . Alternative splicing results in multiple transcript variants, at least one of which encodes a preproprotein that is proteolytically processed. [provided by RefSeq, Jan 2016]

Description

This gene encodes a member of the glycosyl hydrolase 20 family of proteins. The encoded preproprotein is proteolytically processed to generate the alpha subunit of the lysosomal enzyme beta-hexosaminidase. This enzyme, together with the cofactor GM2 activator protein, catalyzes the degradation of the ganglioside GM2, and other molecules containing terminal N-acetyl hexosamines. Mutations in this gene lead to an accumulation of GM2 ganglioside in neurons, the underlying cause of neurodegenerative disorders termed the GM2 gangliosidoses, including Tay-Sachs disease (GM2-gangliosidosis type I). Alternative splicing results in multiple transcript variants, at least one of which encodes a preproprotein that is proteolytically processed. [provided by RefSeq, Jan 2016],

Product Name Alternative

HEXA; Beta-hexosaminidase subunit alpha; Beta-N-acetylhexosaminidase subunit alpha; Hexosaminidase subunit A; N-acetyl-beta-glucosaminidase subunit alpha

UniProt

P06865

Swiss Prot

P06865

Reactivity

Human; Mouse; Rat

Immunogen

Synthesized peptide derived from HEXA . at AA range: 121-170

Target

HEXA

Clonality

Polyclonal

Source

Rabbit

Applications

WB; IHC; IF; ELISA

Concentration

1 mg/ml

Dilution

Western Blot: 1/500 - 1/2000. IHC-p: 1:100-300 ELISA: 1/20000. Not yet tested in other applications.

Buffer

-20°C/1 year

Molecular Weight

60kD

Storage Conditions

-20°C/1 year

Observed Molecular Weight

60kD

Fragment

IgG

Subcellular Location

Lysosome.

Other Product Names

HEXA; Beta-hexosaminidase subunit alpha; Beta-N-acetylhexosaminidase subunit alpha; Hexosaminidase subunit A; N-acetyl-beta-glucosaminidase subunit alpha

Gene ID (Human)

3073

UniProtKB · P06865

Beta-hexosaminidase subunit alpha

HEXA_HUMAN · Homo sapiens

View on UniProt ↗

Primary accession: P06865
Review status: UniProtKB reviewed (Swiss-Prot)
Gene: HEXA
Protein existence: 1: Evidence at protein level
Organism: Homo sapiens (Human)
Taxonomy ID: 9606
Alternative names: —
EC number: 3.2.1.52
Processing: Precursor
Secondary accessions: B4DKE7, E7ENH7, Q53HS8, Q6AI32

Protein keywords

Technical term

3D-structureDirect protein sequencingProteomics identificationReference proteome

Coding sequence diversity

Alternative splicing

Disease

Disease variantGangliosidosisNeurodegeneration

PTM

Disulfide bondGlycoproteinZymogen

Molecular function

GlycosidaseHydrolase

Biological process

Lipid metabolism

Cellular component

Lysosome

Domain

Signal

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