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HEXA rabbit pAb

CAT: 855-ES3963-01Size: 50 µLDry Ice: NoHazardous: No
50 µLSelected
PRICE
AVAILABILITY
24/48H Stock Items & 2 to 6 Weeks non Stock Items.
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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

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