英文名字 Hexosaminidase A (Hexosaminidase Subunit A, HEXA, Beta-hexosaminidase Subunit alpha, Beta-N-acetylhexosaminidase Subunit alpha, MGC99608, N-acetyl-beta-glucosaminidase Subunit alpha, TSD)供应商 MyBioSource产品货号 MBS629852产品报价 ￥询价/0.2mL ￥询价/5x0.2mL ￥询价/其它规格产品说明书 点击...
英文名字 Hexosaminidase A. NT (Hexosaminidase Subunit A, HEXA, Beta-hexosaminidase Subunit alpha, Beta-N-acetylhexosaminidase Subunit alpha, MGC99608, N-acetyl-beta-glucosaminidase Subunit alpha, TSD)供应商 MyBioSource产品货号 MBS624677产品报价 ￥询价/0.2mL ￥询价/5x0.2mL ￥询价/其它规格产品说明书 ...
Human β-hexosaminidase A (Hex A) (αβ) is composed of two subunits whose primary structures are ∼60% identical. Deficiency of either subunit results in severe neurological disease due to the storage of GM2 ganglioside; Tay–Sachs disease, α deficiency, and Sandhoff disease, β deficiency...
When offered as micelles, GD1a-GalNAc was almost not hydrolyzed by Hex A or Hex B, while after incorporation of the ganglioside into the outer leaflet of liposomes, the terminal GalNAc residue was rapidly split off by Hex a. In striking contrast to ganglioside GM2, the major glycolipid ...
A 2.5-year-old Japanese girl who showed signs and symptoms compatible with classic Tay-Sachs disease and had altered β-hexosaminidase B and I 1 as well as completely deficient β-hexosaminidase A activity is reported herein. Heat treatment of the patient's serum and leukocyte samples showed the...
Two benign mutations, C739T(R247W) and C745T(R249W), in the alpha-subunit of beta-hexosaminidase A (Hex A) have been found in all but one of the currently identified Hex A-pseudodeficient subjects. To confirm the relationship of the benign mutations and Hex A pseudodeficiency and to ...
Human lysosomal,β-hexosaminidase exists in two major forms: the A isoform is composed of both α and β chains, while the B form is a homopolymer of β chains. Deficiency of β-hexosaminidase underlies the GM2 gangliosidoses. We have produced active β-hexosamini-dase B in cultured insect...