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Literature summary for 3.4.25.1 extracted from

  • Tang, G.; Perng, M.D.; Wilk, S.; Quinlan, R.; Goldman, J.E.
    Oligomers of mutant glial fibrillary acidic protein (GFAP) inhibit the proteasome system in alexander disease astrocytes, and the small heat shock protein alphaB-crystallin reverses the inhibition (2010), J. Biol. Chem., 285, 10527-10537.
    View publication on PubMedView publication on EuropePMC
Search for more literature for 3.4.25.1

Inhibitors

Inhibitors Comment Organism Structure
glial fibrillary acidic protein human recombinant GFP-tagged, accumulation of the intermediate filament protein, glial fibrillary acidic protein, GFAP, in astrocytes of Alexander disease impairs proteasome function in astrocytes, also oligomers of R239C mutant GFAP inhibit the proteasome system in Alexander disease astrocytes to an even higher extent compared to the wild-type GFAP. The small heat shock protein chaperone alphaB-crystallin reverses the inhibition by shifting the size of the mutant protein from larger oligomers to smaller oligomers and monomers. The proteasome cannot efficiently degrade unassembled R239C GFAP, and the interaction of R239C GFAP with proteasomes actually inhibits proteasomal protease activity in a non-competitive manner, detailed overview Homo sapiens
lactacystin
-
 Homo sapiens
MG132
-
 Homo sapiens

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
-
-

Source Tissue

Source Tissue Comment Organism Textmining
astrocyte from subjects with Alexander disease Homo sapiens
-
white matter from subjects with Alexander disease, significantly decreased PGPH and chymotrypsin-like protease activities in white matter from Alexander disease patients expressing GFAP R239C, R416W, and R239H mutants Homo sapiens
-

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
additional information the proteasome cannot efficiently degrade unassembled R239C GFAP, and the interaction of R239C GFAP with proteasomes actually inhibits proteasomal protease activity. Soluble wild-type GFAP, but not R239C GFAP, is partially degraded by the 20 S proteasome Homo sapiens ?
-
 ?
N-succinyl-LLVY-7-amido-4-methylcoumarin + H2O chymotrypsin-like proteasome activity Homo sapiens N-succinyl-LLVY + 7-amino-4-methylcoumarin
-
 ?
Z-LLE-4-methyl-7-amido-coumarin + H2O peptidylglutamyl peptide hydrolase proteasome activity Homo sapiens Z-LLE + 4-methyl-7-amino-coumarin
-
 ?

Synonyms

Synonyms Comment Organism
20 S proteasome
-
 Homo sapiens
proteasome
-
 Homo sapiens

Temperature Optimum [°C]

Temperature Optimum [°C] Temperature Optimum Maximum [°C] Comment Organism
22
-
 assay at room temperature Homo sapiens

pH Optimum

pH Optimum Minimum pH Optimum Maximum Comment Organism
7.2
-
 assay at Homo sapiens