Matches in SemOpenAlex for { <https://semopenalex.org/work/W2118756200> ?p ?o ?g. }
- W2118756200 endingPage "838" @default.
- W2118756200 startingPage "825" @default.
- W2118756200 abstract "The Hedgehog (Hh) signaling pathway has important functions during metazoan development. The Hh ligand is generated from a precursor by self-cleavage, which requires a free cysteine in the C-terminal part of the protein and results in the production of the cholesterol-modified ligand and a C-terminal fragment. In this paper, we demonstrate that these reactions occur in the endoplasmic reticulum (ER). The catalytic cysteine needs to form a disulfide bridge with a conserved cysteine, which is subsequently reduced by protein disulfide isomerase. Generation of the C-terminal fragment is followed by its ER-associated degradation (ERAD), providing the first example of an endogenous luminal ERAD substrate that is constitutively degraded. This process requires the ubiquitin ligase Hrd1, its partner Sel1, the cytosolic adenosine triphosphatase p97, and degradation by the proteasome. Processing-defective mutants of Hh are degraded by the same ERAD components. Thus, processing of the Hh precursor competes with its rapid degradation, explaining the impaired Hh signaling of processing-defective mutants, such as those causing human holoprosencephaly." @default.
- W2118756200 created "2016-06-24" @default.
- W2118756200 creator A5027336186 @default.
- W2118756200 creator A5028462736 @default.
- W2118756200 creator A5032251246 @default.
- W2118756200 creator A5033968578 @default.
- W2118756200 creator A5036286981 @default.
- W2118756200 creator A5038282147 @default.
- W2118756200 creator A5048192123 @default.
- W2118756200 creator A5072220678 @default.
- W2118756200 creator A5081998200 @default.
- W2118756200 creator A5087757787 @default.
- W2118756200 date "2011-02-28" @default.
- W2118756200 modified "2023-09-28" @default.
- W2118756200 title "Processing and turnover of the Hedgehog protein in the endoplasmic reticulum" @default.
- W2118756200 cites W1481795283 @default.
- W2118756200 cites W1929778619 @default.
- W2118756200 cites W1949772429 @default.
- W2118756200 cites W1964361564 @default.
- W2118756200 cites W1973324969 @default.
- W2118756200 cites W1976551834 @default.
- W2118756200 cites W1981625315 @default.
- W2118756200 cites W1982432510 @default.
- W2118756200 cites W1984136664 @default.
- W2118756200 cites W1990210405 @default.
- W2118756200 cites W1992315987 @default.
- W2118756200 cites W1994620081 @default.
- W2118756200 cites W1995017303 @default.
- W2118756200 cites W1996218285 @default.
- W2118756200 cites W1997889647 @default.
- W2118756200 cites W2007320849 @default.
- W2118756200 cites W2009664225 @default.
- W2118756200 cites W2010206624 @default.
- W2118756200 cites W2015519581 @default.
- W2118756200 cites W2025124261 @default.
- W2118756200 cites W2025226894 @default.
- W2118756200 cites W2026550373 @default.
- W2118756200 cites W2027706325 @default.
- W2118756200 cites W2029119458 @default.
- W2118756200 cites W2033088972 @default.
- W2118756200 cites W2034133901 @default.
- W2118756200 cites W2034277598 @default.
- W2118756200 cites W2034289224 @default.
- W2118756200 cites W2035349696 @default.
- W2118756200 cites W2035535713 @default.
- W2118756200 cites W2042335929 @default.
- W2118756200 cites W2049127225 @default.
- W2118756200 cites W2051881845 @default.
- W2118756200 cites W2056994384 @default.
- W2118756200 cites W2057668553 @default.
- W2118756200 cites W2064609154 @default.
- W2118756200 cites W2065278356 @default.
- W2118756200 cites W2069704514 @default.
- W2118756200 cites W2079261384 @default.
- W2118756200 cites W2079634353 @default.
- W2118756200 cites W2082527135 @default.
- W2118756200 cites W2086462010 @default.
- W2118756200 cites W2095217788 @default.
- W2118756200 cites W2099386409 @default.
- W2118756200 cites W2102248384 @default.
- W2118756200 cites W2105628355 @default.
- W2118756200 cites W2107194582 @default.
- W2118756200 cites W2111415532 @default.
- W2118756200 cites W2111432126 @default.
- W2118756200 cites W2117313136 @default.
- W2118756200 cites W2139577310 @default.
- W2118756200 cites W2145922063 @default.
- W2118756200 cites W2146183177 @default.
- W2118756200 cites W2146394074 @default.
- W2118756200 cites W2146969978 @default.
- W2118756200 cites W2151473091 @default.
- W2118756200 cites W2155946179 @default.
- W2118756200 doi "https://doi.org/10.1083/jcb.201008090" @default.
- W2118756200 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/3051819" @default.
- W2118756200 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/21357747" @default.
- W2118756200 hasPublicationYear "2011" @default.
- W2118756200 type Work @default.
- W2118756200 sameAs 2118756200 @default.
- W2118756200 citedByCount "114" @default.
- W2118756200 countsByYear W21187562002012 @default.
- W2118756200 countsByYear W21187562002013 @default.
- W2118756200 countsByYear W21187562002014 @default.
- W2118756200 countsByYear W21187562002015 @default.
- W2118756200 countsByYear W21187562002016 @default.
- W2118756200 countsByYear W21187562002017 @default.
- W2118756200 countsByYear W21187562002018 @default.
- W2118756200 countsByYear W21187562002019 @default.
- W2118756200 countsByYear W21187562002020 @default.
- W2118756200 countsByYear W21187562002021 @default.
- W2118756200 countsByYear W21187562002022 @default.
- W2118756200 countsByYear W21187562002023 @default.
- W2118756200 crossrefType "journal-article" @default.
- W2118756200 hasAuthorship W2118756200A5027336186 @default.
- W2118756200 hasAuthorship W2118756200A5028462736 @default.
- W2118756200 hasAuthorship W2118756200A5032251246 @default.
- W2118756200 hasAuthorship W2118756200A5033968578 @default.
- W2118756200 hasAuthorship W2118756200A5036286981 @default.
- W2118756200 hasAuthorship W2118756200A5038282147 @default.