ACP2

Lysosomal acid phosphatase has been shown to be deficient in cultured fibroblasts from patients manifesting intermittent vomiting, hypotonia, lethargy, opisthotonos, terminal bleeding and death in early infancy.

The membrane-bound form is converted to the soluble form by sequential proteolytic processing. First, the C-terminal cytoplasmic tail is removed. Cleavage by a lysosomal protease releases the soluble form in the lysosome lumen.

N-glycosylated. The intermediates formed during enzymatic deglycosylation suggest that all eight predicted N-glycosylation sites are used.

Belongs to the histidine acid phosphatase family.

• Lysosome membrane
• Single-pass membrane protein
• Lumenal side
• Lysosome lumen
• The soluble form arises by proteolytic processing of the membrane-bound form.

Lysosomal acid phosphatase, LAP, ACP2

• entrezGene:53
• omim:171650
• swissprot:P11117

Proteins

48344Da