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Invitrogen
HEXB Polyclonal Antibody
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Product Details
PA5-36146
Applications
Tested Dilution
Publications
Product Specifications
Species Reactivity
Human
Published species
Mouse
Host/Isotype
Rabbit
/ IgG
Class
Polyclonal
Type
Antibody
Immunogen
Synthetic peptide corresponding to amino acids 481-530 of human Hexb
Conjugate
Form
Liquid
Concentration
1 mg/mL
Amount
100 µg
Purification
Antigen affinity chromatography
Storage buffer
PBS, pH 7.2, with 50% glycerol
Contains
0.02% sodium azide
Storage conditions
Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles.
Shipping conditions
Ambient (domestic); Wet ice (international)
RRID
Product Specific Information
This antibody detects endogenous protein at a molecular weight of 63 kDa.
Purity is >95% by SDS-PAGE.
Target Information
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.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
Bioinformatics
Protein Aliases: beta-hexosaminidase beta-subunit; Beta-hexosaminidase subunit beta; Beta-N-acetylhexosaminidase subunit beta; Cervical cancer proto-oncogene 7 protein; epididymis luminal protein 248; epididymis secretory protein Li 111; epididymis secretory sperm binding protein; HCC-7; hexosaminidase B (beta polypeptide); Hexosaminidase subunit B; N-acetyl-beta-glucosaminidase prepro-polypeptide; N-acetyl-beta-glucosaminidase subunit beta
Gene Aliases: ENC-1AS; HCC7; HEL-248; HEL-S-111; HEXB
UniProt ID: (Human) P07686
Entrez Gene ID: (Human) 3074
hydrolase activity, hydrolyzing O-glycosyl compounds
beta-N-acetylhexosaminidase activity
protein binding
acetylglucosaminyltransferase activity
hexosaminidase activity
beta-N-acetylglucosaminidase activity
hydrolase activity
hydrolase activity, acting on glycosyl bonds
carbohydrate binding
identical protein binding
macromolecular complex binding
skeletal system development
carbohydrate metabolic process
N-acetylglucosamine metabolic process
N-glycan processing
lipid metabolic process
glycosphingolipid metabolic process
ganglioside catabolic process
cellular calcium ion homeostasis
lysosome organization
single fertilization
penetration of zona pellucida
sensory perception of sound
locomotory behavior
male courtship behavior
regulation of cell shape
phospholipid biosynthetic process
oligosaccharide catabolic process
regulation of metabolic process
lipid storage
sexual reproduction
glycosaminoglycan metabolic process
chondroitin sulfate catabolic process
dermatan sulfate catabolic process
hyaluronan catabolic process
myelination
astrocyte cell migration
positive regulation of transcription from RNA polymerase II promoter
oogenesis
neuromuscular process controlling balance
neuromuscular process
neuron cellular homeostasis
carbohydrate derivative metabolic process
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Performance Guarantee
If an Invitrogen™ antibody doesn't perform as described on our website or datasheet,we'll replace the product at no cost to you, or provide you with a credit for a future purchase.*
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