Hexosaminidase A 抗体 (Middle Region) (APC)
Our Local Distributor
北京 101111
Quick Overview for Hexosaminidase A 抗体 (Middle Region) (APC) (ABIN7997493)
抗原
See all Hexosaminidase A (HEXA) 抗体适用
宿主
克隆类型
标记
应用范围
-
-
抗原表位
- Middle Region
-
原理
- Anti-HEXA Antibody APC Conjugated
-
特异性
- No cross reactivity with other proteins.
-
交叉反应 (详细)
- No cross-reactivity with other proteins
-
预测反应
- different from the related rat,mouse sequences by one amino acid.
-
纯化方法
- Immunogen affinity purified.
-
免疫原
- A synthetic peptide corresponding to a sequence in the middle region of human HEXA, different from the related rat and mouse sequences by one amino acid.
-
亚型
- IgG
-
-
-
-
应用备注
- Flow Cytometry, Optimal dilutions should be determined by end users.
-
限制
- 仅限研究用
-
-
-
状态
- Liquid
-
缓冲液
- Each vial contains 50 % glycerol, 0.9 % NaCl, 0.2 % Na2HPO4, 0.02 % Sodium azide.
-
储存液
- Sodium azide
-
注意事项
- This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
-
储存条件
- -20 °C
-
储存方法
- At -20°C for one year from date of receipt. Avoid repeated freezing and thawing. Protect from light.
-
有效期
- 12 months
-
-
- Hexosaminidase A (HEXA)
-
别名
- HEXA
-
背景
-
Background: HEXA (hexosaminidase A (alpha polypeptide)) is an enzyme that in humans is encoded by the HEXA gene. Hexosaminidase A and the cofactor GM2 activator protein catalyze the degradation of the GM2 gangliosides and other molecules containing terminal N-acetyl hexosamines The HEXA gene encodes the alpha subunit of hexosaminidase A, a lysosomal enzyme involved in the breakdown of gangliosides. The HEXA gene is mapped on 15q23. Even though the alpha and beta subunits of hexosaminidase A can both cleave GalNAc residues, only the alpha subunit is able to hydrolyze GM2 gangliosides. The alpha subunit contains a key residue, Arg-424, which is essential for binding the N-acetyl-neuramanic residue of GM2 gangliosides. Chimeric constructs were expressed in HeLa cells and selected constructs were produced in the baculovirus expression system to determine their ability to degrade GM2 ganglioside in the presence of GM2 activator protein. Their results allowed them to define 2 noncontiguous sequences in the alpha subunit (amino acids 1-191 and 403-529) which, when substituted into analogous positions in the beta subunit, conferred activity against the sulfated substrate.
Gene Full Name: hexosaminidase subunit alpha
Sequence Similarities: Belongs to the glycosyl hydrolase 20 family.
-
基因ID
- 3073
-
UniProt
- P06865
-
途径
- Sensory Perception of Sound, Glycosaminoglycan Metabolic Process
抗原
-