Lysosomal hydrolases are synthesized in the rough endoplasmic reticulum and specifically
transported through the Golgi apparatus to the trans-Golgi network, from which transport …

Lysosomal storage disorders comprise a group of more than 30 different diseases. The
common feature of these diseases is the deficiency of a lysosomal protein that is part of a …

Cathepsin D (EC 3.4. 23.5) is a lysosomal protease suspected to play important roles in
protein catabolism, antigen processing, degenerative diseases, and breast cancer …

The acid sphingomyelinase (ASM) gene, which has been implicated in ceramide-mediated
cell signaling and atherogenesis, gives rise to both lysosomal SMase (L-SMase), which is …

In humans, two major β-hexosaminidase isoenzymes exist: Hex A and Hex B. Hex A is a
heterodimer of subunits α and β (60% identity), whereas Hex B is a homodimer of β …

Mammalian cells express two different mannose 6-phosphate receptors (MPR 46 and MPR
300), which both mediate targeting of Man-6-P-containing lysosomal proteins to lysosomes …

Two crystal structures are described for the lysosomal aspartic protease cathepsin D (EC
3.4. 23.5). The molecular replacement method was used with X‐ray diffraction data to 3 A …

UDP-N-acetylglucosamine: lysosomal-enzyme N-acetylglucosamine-1-phosphotransferase
(GlcNAc-phosphotransferase) catalyzes the initial step in the synthesis of the mannose 6 …

Cathepsin S (CTSS), a lysosomal protease, belongs to a family of cysteine cathepsin
proteases that promote degradation of damaged proteins in the endolysosomal pathway …

In higher eukaryotes, the transport of soluble lysosomal enzymes involves the recognition of
their mannose 6‐phosphate signal by two receptors: the cation‐independent mannose 6 …