HEXB Rabbit Polyclonal Antibody

Specifications

Product Data
Applications	WB
Recommend Dilution	WB: 1:500~1:1000 IHC: 1:50~1:200
Reactivity	Human
Host	Rabbit
Clonality	Polyclonal
Immunogen	Synthetic peptide, corresponding to amino acids 481-530 of Human Hexb.
Specificity	Hexb (S516) polyclonal antibody detects endogenous levels of Hexb protein.
Formulation	Rabbit IgG, 1mg/ml in PBS with 0.02% sodium azide, 50% glycerol, pH7.2
Concentration	1mg/ml
Conjugation	Unconjugated
Storage Condition	Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.
Predicted Protein Size	~ 63 kDa
Gene Name	hexosaminidase subunit beta
Database Link	Entrez Gene 3074 Human UniProt ID P07686
Background	Hexosaminidase B (HEXB), also designated beta-hexosaminidase B, is a Hexosaminidase B (HEXB), also designated b-hexosaminidase B, is a tetramer of two b-A and two b-B chains and is found in the lysosomes of cells. Sandhoff disease (SD), also known as GM2-gangliosidosis type II, is caused by mutations in the HEXB gene that affect the b subunit. These mutations disrupt the activity of HEXB and HEXA, which prevents the breakdown of GM2 ganglioside, a fatty material found in the brain, therby rendering both the HEXA and HEXB enzymes deficient. SD is a rare autosomal recessive disorder characterized by an accumulation of GM2 ganglioside, which causes progressive destruction of the central nervous system. Sandhoff disease is similar to Tay-Sachs disease, which is caused by mutations in the HEXA gene, although SD is more severe.
Synonyms	Beta-hexosaminidase subunit beta; Beta-N-acetylhexosaminidase subunit beta; Cervical cancer proto-oncogene 7 protein; HCC-7; HCC7; HEXB; Hexosaminidase subunit B; N-acetyl-beta-glucosaminidase subunit beta
Note	For research use only, not for use in diagnostic procedure.
Reference Data

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