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安全信息

SAB1402803

Sigma-Aldrich

Monoclonal Anti-HEXA antibody produced in mouse

clone 3D1, purified immunoglobulin, buffered aqueous solution

别名:

MGC99608, TSD

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About This Item

UNSPSC代码:
12352203
NACRES:
NA.41

生物来源

mouse

质量水平

偶联物

unconjugated

抗体形式

purified immunoglobulin

抗体产品类型

primary antibodies

克隆

3D1, monoclonal

形式

buffered aqueous solution

分子量

antigen ~84.3 kDa

种属反应性

human

技术

immunofluorescence: suitable
indirect ELISA: suitable
western blot: 1-5 μg/mL

同位素/亚型

IgG1κ

NCBI登记号

UniProt登记号

运输

dry ice

储存温度

−20°C

靶向翻译后修饰

unmodified

基因信息

human ... HEXA(3073)

一般描述

Hexosaminidase subunit alpha (HEXA) gene is located on the human chromosome 15q23-q24. HEXA is found to be associated with the external leaflet of plasma membrane of human fibroblasts. α and β subunits of HEXA have 57% of similarity in their amino acid sequences.

免疫原

HEXA (AAH18927, 1 a.a. ~ 529 a.a) full-length recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.

Sequence
MTSSRLWFSLLLAAAFAGRATALWPWPQNFQTSDQRYVLYPNNFQFQYDVSSAAQPGCSVLDEAFQRYRDLLFGSGSWPRPYLTGKRHTLEKNVLVVSVVTPGCNQLPTLESVENYTLTINDDQCLLLSETVWGALRGLETFSQLVWKSAEGTFFINKTEIEDFPRFPHRGLLLDTSRHYLPLSSILDTLDVMAYNKLNVFHWHLVDDPSFPYESFTFPELMRKGSYNPVTHIYTAQDVKEVIEYARLRGIRVLAEFDTPGHTLSWGPGIPGLLTPCYSGSEPSGTFGPVNPSLNNTYEFMSTFFLEVSSVFPDFYLHLGGDEVDFTCWKSNPEIQDFMRKKGFGEDFKQLESFYIQTLLDIVSSYGKGYVVWQEVFDNKVKIQPDTIIQVWREDIPVNYMKELELVTKAGFRALLSAPWYLNRISYGPDWKDFYVVEPLAFEGTPEQKALVIGGEACMWGEYVDNTNLVPRLWPRAGAVAERLWSNKLTSDLTFAYERLSHFRCELLRRGVQAQPLNVGFCEQEFEQT

生化/生理作用

Hexosaminidase subunit alpha (HEXA) degrades the carbohydrate moieties of glycoproteins, glycolipids and proteoglycans. Mutations in the α chain of HEXA impairs folding, trafficking and enzyme assembly. Lysosomal β-hexosaminidase α subunit is downregulated in prostate cancer cells due to hypermethylation of gene promoter.

外形

Solution in phosphate buffered saline, pH 7.4

免责声明

Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

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WGK

WGK 1

闪点(°F)

Not applicable

闪点(°C)

Not applicable

法规信息

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The X-ray crystal structure of human $\beta$-hexosaminidase B provides new insights into Sandhoff disease
Maier T, et al.
Journal of Molecular Biology, 328(3), 669-681 (2003)
Identification of plasma membrane associated mature beta-hexosaminidase A, active towards GM2 ganglioside, in human fibroblasts
Mencarelli S, et al.
Febs Letters, 579(25), 5501-5506 (2005)
Tay-Sachs disease mutations in HEXA target the alpha chain of hexosaminidase A to endoplasmic reticulum-associated degradation
Dersh D, et al.
Molecular Biology of the Cell, 27(24), 3813-3827 (2016)
Production of recombinant beta-hexosaminidase A, a potential enzyme for replacement therapy for Tay-Sachs and Sandhoff diseases, in the methylotrophic yeast Ogataea minuta
Akeboshi H, et al.
Applied and Environmental Microbiology, 73(15), 4805-4812 (2007)
Hypermethylation contributes to down-regulation of lysosomal beta-hexosaminidase alpha subunit in prostate cancer cells
Costanzi E, et al.
Biochimie, 101, 75-82 (2014)

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