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Product Details
16229-1-AP
Applications
Tested Dilution
Publications
Product Specifications
Species Reactivity
Human,
Mouse,
Rat
Published species
Mouse
Host/Isotype
Rabbit
/ IgG
Class
Polyclonal
Type
Antibody
Immunogen
HEXB Fusion Protein Ag8683 (1-332 aa encoded by BC017378)
Conjugate
Form
Liquid
Concentration
0.35 mg/mL
Purification
Antigen affinity chromatography
Storage buffer
PBS, pH 7.3, with 50% glycerol
Contains
0.02% sodium azide
Storage conditions
-20°C
Shipping conditions
Wet ice (domestic); Dry ice (international)
Product Specific Information
Immunogen sequence: MELCGLGLP RPPMLLALLL ATLLAAMLAL LTQVALVVQV AEAARAPSVS AKPGPALWPL PLSVKMTPNL LHLAPENFYI SHSPNSTAGP SCTLLEEAFR RYHGYIFGFY KWHHEPAEFQ AKTQVQQLLV SITLQSECDA FPNISSDESY TLLVKEPVAV LKANRVWGAL RGLETFSQLV YQDSYGTFTI NESTIIDSPR FSHRGILIDT SRHYLPVKII LKTLDAMAFN KFNVLHWHIV DDQSFPYQSI TFPELSNKGS YSLSHVYTPN DVRMVIEYAR LRGIRVLPEF DTPGHTLSWG KGQKDLLTPC YSRQNKLDSF GPINPTLNTT YSF (1-332 aa encoded by BC017378)
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 subunit beta; Beta-N-acetylhexosaminidase subunit beta; Cervical cancer proto-oncogene 7 protein; epididymis luminal protein 248; epididymis secretory protein Li 111; HCC-7; hexosaminidase B (beta polypeptide); Hexosaminidase subunit B; N-acetyl-beta-glucosaminidase subunit beta
Gene Aliases: ENC-1AS; HCC7; HEL-248; HEL-S-111; HEXB
UniProt ID: (Human) P07686, (Mouse) P20060, (Rat) Q6AXR4
Entrez Gene ID: (Human) 3074, (Mouse) 15212, (Rat) 294673
beta-N-acetylhexosaminidase activity
acetylglucosaminyltransferase activity
protein homodimerization activity
protein heterodimerization activity
hydrolase activity, hydrolyzing O-glycosyl compounds
hexosaminidase activity
beta-N-acetylglucosaminidase activity
hydrolase activity
hydrolase activity, acting on glycosyl bonds
carbohydrate binding
glycosidase
skeletal system development
glycosphingolipid metabolic process
ganglioside catabolic process
cellular calcium ion homeostasis
lysosome organization
penetration of zona pellucida
sensory perception of sound
locomotory behavior
male courtship behavior
regulation of cell shape
phospholipid biosynthetic process
oligosaccharide catabolic process
lipid storage
chondroitin sulfate catabolic process
hyaluronan catabolic process
keratan sulfate catabolic process
myelination
astrocyte cell migration
cellular protein metabolic process
positive regulation of transcription from RNA polymerase II promoter
oogenesis
neuromuscular process controlling balance
carbohydrate metabolic process
N-acetylglucosamine metabolic process
single fertilization
metabolic process
sexual reproduction
glycosaminoglycan metabolic process
regulation of cellular metabolic process
neuromuscular process
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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