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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
PA5-79359
产品规格
种属反应
Human
宿主/亚型
Rabbit
/ IgG
分类
Polyclonal
类型
Antibody
抗原
E.coli-derived human HEXB recombinant protein (Position: K381-M556).
偶联物
Unconjugated
Unconjugated
Unconjugated
形式
Lyophilized
浓度
500 µg/mL
规格
100 µg
纯化类型
Antigen affinity chromatography
保存液
PBS with 5mg BSA
内含物
0.05mg sodium azide
保存条件
-20°C
运输条件
Ambient (domestic); Wet ice (international)
RRID
产品详细信息
Reconstitute with 0.2 mL of distilled water to yield a concentration of 500 µg/mL.
Positive Control - WB: human U87 whole cell. IHC: Human Lung Cancer Tissue.
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 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
Entrez Gene ID: (Human) 3074
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