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Invitrogen
HEXB Polyclonal Antibody
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Please note: We are reviewing Western blot images included in the antibody testing data in our catalog, including those provided by third parties. Unless expressly labeled or annotated as “raw-unedited”, Western blot images included in the antibody testing data in our catalog may have been edited, optimized or otherwise adjusted for presentation.
产品信息
PA5-101082
应用
建议稀释比
已发表文章
产品规格
种属反应
Human,
Mouse,
Rat
已发表种属
Mouse
宿主/亚型
Rabbit
/ IgG
分类
Polyclonal
类型
Antibody
抗原
A synthesized peptide derived from human HEXB(Accession P07686), corresponding to amino acid residues T496-L546.
偶联物
Unconjugated
Unconjugated
Unconjugated
形式
Liquid
浓度
1 mg/mL
规格
100 µg
纯化类型
Affinity chromatography
保存液
PBS, pH 7.4, with 50% glycerol
内含物
0.02% sodium azide
保存条件
-20°C
运输条件
Ambient (domestic); Wet ice (international)
RRID
产品详细信息
Antibody detects endogenous levels of total HEXB.
靶标信息
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.
仅用于科研。不用于诊断过程。未经明确授权不得转售。
生物信息学
蛋白别名: Beta-hexosaminidase subunit beta; Beta-N-acetylhexosaminidase subunit beta; Cervical cancer proto-oncogene 7 protein; HCC-7; Hexosaminidase subunit B; N-acetyl-beta-glucosaminidase subunit beta
基因别名: HCC7; HEXB
UniProt ID: (Mouse) P20060, (Rat) Q6AXR4
Entrez Gene ID: (Mouse) 15212, (Rat) 294673
hydrolase activity, hydrolyzing O-glycosyl compounds
beta-N-acetylhexosaminidase activity
hexosaminidase activity
beta-N-acetylglucosaminidase activity
hydrolase activity
hydrolase activity, acting on glycosyl bonds
carbohydrate binding
protein homodimerization activity
protein heterodimerization activity
glycosidase
skeletal system development
carbohydrate metabolic process
N-acetylglucosamine 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
metabolic process
regulation of cell shape
phospholipid biosynthetic process
oligosaccharide catabolic process
lipid storage
sexual reproduction
glycosaminoglycan metabolic process
regulation of cellular metabolic process
myelination
astrocyte cell migration
cellular protein metabolic process
positive regulation of transcription from RNA polymerase II promoter
oogenesis
neuromuscular process controlling balance
neuromuscular process
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