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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
PA5-63123
产品规格
种属反应
Human
宿主/亚型
Rabbit
/ IgG
分类
Polyclonal
类型
Antibody
抗原
偶联物
Unconjugated
Unconjugated
Unconjugated
形式
Liquid
浓度
0.1 mg/mL
规格
10 µg
纯化类型
Antigen affinity chromatography
保存液
PBS, pH 7.2, with 40% glycerol
内含物
0.02% sodium azide
保存条件
Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles.
运输条件
Ambient (domestic); Wet ice (international)
RRID
产品详细信息
Immunogen sequence: PSVSAKPGPA LWPLPLSVKM TPNLLHLAPE NFYISHSPNS TAGPSCTLLE EAFRRYHGYI
Highest antigen sequence identity to the following orthologs: Mouse - 60%, Rat - 60%.
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。
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生物信息学
蛋白别名: beta-hexosaminidase beta-subunit; 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
基因别名: ENC-1AS; HEL-248; HEL-S-111
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
carbohydrate binding
identical protein binding
macromolecular complex binding
carbohydrate metabolic process
N-acetylglucosamine metabolic process
ganglioside catabolic process
single fertilization
regulation of cell shape
lipid catabolic process
glycosaminoglycan metabolic process
chondroitin sulfate catabolic process
dermatan sulfate catabolic process
hyaluronan catabolic process
astrocyte cell migration
positive regulation of transcription from RNA polymerase II promoter
carbohydrate derivative metabolic process
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