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Literature summary for 3.1.6.8 extracted from

  • Poeppel, P.; Abouzied, M.M.; Voelker, C.; Gieselmann, V.
    Misfolded endoplasmic reticulum retained subunits cause degradation of wild-type subunits of arylsulfatase A heteromers (2010), FEBS J., 277, 3404-3414.
    View publication on PubMed
Search for more literature for 3.1.6.8

Cloned(Commentary)

Cloned (Comment) Organism
coexpression and secretion of misfolded, enzymatically inactive enzyme with the wild-type enzyme, whose activity is diminished upon coexpression with misfolded ASA polypeptides, in BHK cells Homo sapiens

Protein Variants

Protein Variants Comment Organism
D255H inactive misfolded mutant D255H-ASA Homo sapiens
D335V inactive misfolded mutant D335V-ASA Homo sapiens
G86D inactive misfolded mutant G86D-ASA Homo sapiens
additional information secretion and half-life of wild-type enzyme are reduced upon co-expression of defective P377L-pdASA Homo sapiens
P136L inactive misfolded mutant P136LASA Homo sapiens
P377L inactive misfolded mutant P377L-pdASA Homo sapiens
T201C inactive misfolded mutant T201C-ASA Homo sapiens
T275M inactive misfolded mutant T275M-ASA Homo sapiens

Inhibitors

Inhibitors Comment Organism Structure
additional information secretion and stability of wtASA is decreased by the coexpression of defective P377L-pdASA Homo sapiens

Localization

Localization Comment Organism GeneOntology No. Textmining
lysosome
-
 Homo sapiens 5764
-

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
-
-

Source Tissue

Source Tissue Comment Organism Textmining
additional information the polypeptide complexes, rather than the monomers, are subject to endoplasmic reticulum quality control and, within a heteromer, the misfolded subunit exerts a dominant negative effect on the wild-type subunit Homo sapiens
-

Subunits

Subunits Comment Organism
dimer mature enzyme Homo sapiens
More mature lysosomal arylsulfatase A forms dimers at neutral pH, while in the early biosynthetic pathway, arylsulfatase A forms oligomers with more than two subunits. Within a heteromer, the misfolded subunit exerts a dominant negative effect on the wild-type subunit Homo sapiens
oligomer early enzyme Homo sapiens

Synonyms

Synonyms Comment Organism
arylsulfatase A
-
 Homo sapiens
ASA
-
 Homo sapiens

General Information

General Information Comment Organism
additional information heteromerization of wild-type and misfolded endoplasmic reticulum-degraded arylsulfatase A polypeptides affects the quality control of wild-type arylsulfatase A subunits. Within a heteromer, the misfolded subunit exerts a dominant negative effect on the wild-type subunit. Mechanism, overview Homo sapiens