2of5

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Template:STRUCTURE 2of5

Contents

Oligomeric Death Domain complex

Template:ABSTRACT PUBMED 17289572

Disease

[CRADD_HUMAN] Defects in CRADD are the cause of mental retardation autosomal recessive type 34 (MRT34) [MIM:614499]. A disorder characterized by significantly below average general intellectual functioning associated with impairments in adaptative behavior and manifested during the developmental period. MRT34 is a non-syndromic form. Affected individuals have mildly delayed development and significantly impaired cognitive function, precluding independent living and self-care. Speech is rudimentary, but articulate; autism is not present.[1]

Function

[CRADD_HUMAN] Apoptotic adaptor molecule specific for caspase-2 and FASL/TNF receptor-interacting protein RIP. In the presence of RIP and TRADD, CRADD recruits caspase-2 to the TNFR-1 signalling complex. [PIDD_HUMAN] Promotes apoptosis downstream of the tumor suppressor as component of the DNA damage/stress response pathway that connects p53/TP53 to apoptosis. Associates with NEMO/IKBKG and RIP1 and enhances sumoylation and ubiquitination of NEMO/IKBKG which is important for activation of the transcription factor NF-kappa-B. Associates with CASP2/caspase-2 and CRADD/RAIDD, and induces activation of CASP2 which an important regulator in apoptotic pathways.[2] [3] [4]

About this Structure

2of5 is a 12 chain structure with sequence from Homo sapiens. Full crystallographic information is available from OCA.

Reference

  • Park HH, Logette E, Raunser S, Cuenin S, Walz T, Tschopp J, Wu H. Death domain assembly mechanism revealed by crystal structure of the oligomeric PIDDosome core complex. Cell. 2007 Feb 9;128(3):533-46. PMID:17289572 doi:10.1016/j.cell.2007.01.019
  1. ↑ Puffenberger EG, Jinks RN, Sougnez C, Cibulskis K, Willert RA, Achilly NP, Cassidy RP, Fiorentini CJ, Heiken KF, Lawrence JJ, Mahoney MH, Miller CJ, Nair DT, Politi KA, Worcester KN, Setton RA, Dipiazza R, Sherman EA, Eastman JT, Francklyn C, Robey-Bond S, Rider NL, Gabriel S, Morton DH, Strauss KA. Genetic mapping and exome sequencing identify variants associated with five novel diseases. PLoS One. 2012;7(1):e28936. doi: 10.1371/journal.pone.0028936. Epub 2012 Jan 17. PMID:22279524 doi:10.1371/journal.pone.0028936
  2. ↑ Lin Y, Ma W, Benchimol S. Pidd, a new death-domain-containing protein, is induced by p53 and promotes apoptosis. Nat Genet. 2000 Sep;26(1):122-7. PMID:10973264 doi:10.1038/79102
  3. ↑ Tinel A, Tschopp J. The PIDDosome, a protein complex implicated in activation of caspase-2 in response to genotoxic stress. Science. 2004 May 7;304(5672):843-6. Epub 2004 Apr 8. PMID:15073321 doi:10.1126/science.1095432
  4. ↑ Janssens S, Tinel A, Lippens S, Tschopp J. PIDD mediates NF-kappaB activation in response to DNA damage. Cell. 2005 Dec 16;123(6):1079-92. PMID:16360037 doi:S0092-8674(05)01042-1

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