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Proteintech
HEXA Polyclonal Antibody
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产品信息
11317-1-AP
应用
建议稀释比
已发表文章
产品规格
已发表种属
Mouse
宿主/亚型
Rabbit
/ IgG
分类
Polyclonal
类型
Antibody
抗原
HEXA Fusion Protein Ag1854 (1-529 aa encoded by BC018927)
偶联物
形式
Liquid
浓度
0.15 mg/mL
保存条件
-20°C
运输条件
Wet ice (domestic); Dry ice (international)
产品详细信息
The antibody, 11317-1-AP, can recognize both alpha and beta subunit of beta-hexosaminidase. The mature alpha subunit is composed of a major 54 kDa polypeptide and a minor 6 kDa polypeptide. The mature beta-subunit originates from its precursor by two proteolytic cleavage events forming 3 chains: 11 kDa, 24 kDa, and 28 kDa.
Immunogen sequence: MTSSRLWFS LLLAAAFAGR ATALWPWPQN FQTSDQRYVL YPNNFQFQYD VSSAAQPGCS VLDEAFQRYR DLLFGSGSWP RPYLTGKRHT LEKNVLVVSV VTPGCNQLPT LESVENYTLT INDDQCLLLS ETVWGALRGL ETFSQLVWKS AEGTFFINKT EIEDFPRFPH RGLLLDTSRH YLPLSSILDT LDVMAYNKLN VFHWHLVDDP SFPYESFTFP ELMRKGSYNP VTHIYTAQDV KEVIEYARLR GIRVLAEFDT PGHTLSWGPG IPGLLTPCYS GSEPSGTFGP VNPSLNNTYE FMSTFFLEVS SVFPDFYLHL GGDEVDFTCW KSNPEIQDFM RKKGFGEDFK QLESFYIQTL LDIVSSYGKG YVVWQEVFDN KVKIQPDTII QVWREDIPVN YMKELELVTK AGFRALLSAP WYLNRISYGP DWKDFYVVEP LAFEGTPEQK ALVIGGEACM WGEYVDNTNL VPRLWPRAGA VAERLWSNKL TSDLTFAYER LSHFRCELLR RGVQAQPLNV GFCEQEFEQT (1-529 aa encoded by BC018927)
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).
仅用于科研。不用于诊断过程。未经明确授权不得转售。
