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Complete localization of disulfide bonds in GM2 activator protein
Lysosomal degradation of ganglioside GM2 by hexosaminidase A requires the presence of a small, non‐enzymatic cofactor, the GM2‐activator protein (GM2AP). Lack of functional protein leads to the ABExpand
Molecular analysis of a GM2-activator deficiency in two patients with GM2-gangliosidosis AB variant.
Pulse/chase labeling of cultured fibroblasts of the patients, in presence and absence of brefeldin A, indicates a premature degradation of both--mutant and truncated--GM2APs in the endoplasmic reticulum or Golgi. Expand
Photoaffinity labeling of human lysosomal beta-hexosaminidase B. Identification of Glu-355 at the substrate binding site.
The carbene precursor 3-azi-1-[([6-3H]-2-acetamido-2-deoxy-1-beta-D-galactopyranosyl)thi o -butane has been used as a photoaffinity label for human lysosomal beta-hexosaminidase B (Hex B, EC purified to apparent homogeneity from postmortal liver. Expand
Purification of acid sphingomyelinase from human placenta: Characterization and N‐terminal sequence
Human placental acid sphingomyelinase (ASM) was purified by sequential chromatography employing polyclonal anti‐ASM antibodies and microsequencing revealed the N‐terminal amino acid sequence of the mature placental enzyme. Expand
Expression of the Three Alternative Forms of the Sphingolipid Activator Protein Precursor in Baby Hamster Kidney Cells and Functional Assays in a Cell Culture System (*)
It is established that all three forms of the SAP-B can function as sulfatide/globotriaosylceramide activator. Expand
Biosynthesis, Processing, and Intracellular Transport of GM2 Activator Protein in Human Epidermal Keratinocytes
It is concluded that a mannose-6-phosphate-independent pathway for the lysosomal delivery of GM2 AP exists in cultured human keratinocytes and is even more effective for nonglycosylated GM2AP. Expand
Iridoid biosynthesis in staphylinid rove beetles (Coleoptera: Staphylinidae, Philonthinae).
A detailed account of iridoid biosynthesis in rove beetles is constructed, which resembles the biosynthetic route in leaf beetle larvae, but exhibits distinct stereochemical differences. Expand
Photoaffinity Labeling of Human Lysosomal β-Hexosaminidase B
The carbene precursor 3-azi-1-[([6-3H]-2-acetamido-2-deoxy-1-β-D-galactopyranosyl)thio]-butane (also designated [3H]-1-ATB-GalNAc) has been used as a photoaffinity label for human lysosomalExpand