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产品信息
PA5-79357
产品规格
种属反应
Human,
Rat
宿主/亚型
Rabbit
/ IgG
分类
Polyclonal
类型
Antibody
抗原
A synthetic peptide corresponding to a sequence in the middle region of human HEXA (191-207aa DVMAYNKLNVFHWHLVD).
偶联物
形式
Lyophilized
浓度
500 µg/mL
纯化类型
Antigen affinity chromatography
保存液
PBS with 5mg BSA
内含物
0.05mg sodium azide, 0.05mg thimerosal
保存条件
-20°C
运输条件
Ambient (domestic); Wet ice (international)
RRID
AB_2746473
产品详细信息
Reconstitute with 0.2 mL of distilled water to yield a concentration of 500 µg/mL.
靶标信息
This gene encodes the alpha subunit of the lysosomal enzyme beta-hexosaminidase that, together with the cofactor GM2 activator protein, catalyzes the degradation of the ganglioside GM2, and other molecules containing terminal N-acetyl hexosamines. Beta-hexosaminidase is composed of two subunits, alpha and beta, which are encoded by separate genes. Both beta-hexosaminidase alpha and beta subunits are members of family 20 of glycosyl hydrolases. Mutations in the alpha or beta subunit genes lead to an accumulation of GM2 ganglioside in neurons and neurodegenerative disorders termed the GM2 gangliosidoses. Alpha subunit gene mutations lead to Tay-Sachs disease (GM2-gangliosidosis type I).
⚠WARNING: This product can expose you to chemicals including mercury, which is known to the State of California to cause birth defects or other reproductive harm. For more information go to www.P65Warnings.ca.gov.
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 alpha; Beta-N-acetylhexosaminidase subunit alpha; EC 3.2.1; EC 3.2.1.52; hexosaminidase A (alpha polypeptide); Hexosaminidase subunit A; Hexose aminidase A (alpha polypeptide); MGC99608; N-acetyl-beta-glucosaminidase subunit alpha
基因别名: HEXA; TSD
UniProt ID: (Human) P06865, (Rat) Q641X3
Entrez Gene ID: (Human) 3073, (Rat) 300757
carbohydrate metabolic process
glycosaminoglycan biosynthetic process
glycosphingolipid metabolic process
chondroitin sulfate catabolic process
hyaluronan catabolic process
keratan sulfate catabolic process
skeletal system development
ganglioside catabolic process
lysosome organization
sensory perception of sound
locomotory behavior
adult walking behavior
lipid storage
sexual reproduction
glycosaminoglycan metabolic process
myelination
cell morphogenesis involved in neuron differentiation
neuromuscular process controlling posture
neuromuscular process controlling balance
SMAD protein signal transduction
