3cki: Difference between revisions

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~~[[Image:3cki.png|left|200px]]~~

===Crystal structure of the TACE-N-TIMP-3 complex===

===~~Crystal structure~~ of the ~~TACE-N-TIMP-3 complex===~~

==Disease==

[[http://www.uniprot.org/uniprot/ADA17_HUMAN ADA17_HUMAN]] Defects in ADAM17 are a cause of neonatal inflammatory skin and bowel disease (NISBD) [MIM:[http://omim.org/entry/614328 614328]]. NISBD is a disorder characterized by inflammatory features with neonatal onset, involving the skin, hair, and gut. The skin lesions involve perioral and perianal erythema, psoriasiform erythroderma, with flares of erythema, scaling, and widespread pustules. Gastrointestinal symptoms include malabsorptive diarrhea that is exacerbated by intercurrent gastrointestinal infections. The hair is short or broken, and the eyelashes and eyebrows are wiry and disorganized.<ref>PMID:22010916</ref> [[http://www.uniprot.org/uniprot/TIMP3_HUMAN TIMP3_HUMAN]] Defects in TIMP3 are the cause of Sorsby fundus dystrophy (SFD) [MIM:[http://omim.org/entry/136900 136900]]. SFD is a rare autosomal dominant macular disorder with an age of onset in the fourth decade. It is characterized by loss of central vision from subretinal neovascularization and atrophy of the ocular tissues. Generally, macular disciform degeneration develops in the patients eye within 6 months to 6 years.<ref>PMID:7894485</ref><ref>PMID:8634721</ref><ref>PMID:7550309</ref><ref>PMID:8728699</ref><ref>PMID:8981947</ref>

~~{{ABSTRACT_PUBMED_18638486}}~~

==Function==

[[http://www.uniprot.org/uniprot/ADA17_HUMAN ADA17_HUMAN]] Cleaves the membrane-bound precursor of TNF-alpha to its mature soluble form. Responsible for the proteolytical release of soluble JAM3 from endothelial cells surface. Responsible for the proteolytic release of several other cell-surface proteins, including p75 TNF-receptor, interleukin 1 receptor type II, p55 TNF-receptor, transforming growth factor-alpha, L-selectin, growth hormone receptor, MUC1 and the amyloid precursor protein. Also involved in the activation of Notch pathway (By similarity).<ref>PMID:12441351</ref><ref>PMID:20592283</ref> [[http://www.uniprot.org/uniprot/TIMP3_HUMAN TIMP3_HUMAN]] Complexes with metalloproteinases (such as collagenases) and irreversibly inactivates them by binding to their catalytic zinc cofactor. May form part of a tissue-specific acute response to remodeling stimuli. Known to act on MMP-1, MMP-2, MMP-3, MMP-7, MMP-9, MMP-13, MMP-14 and MMP-15.

==About this Structure==

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==Reference==

[[Category: ADAM 17 endopeptidase]]

[[Category: Homo sapiens]]

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-[[Image:3cki.png|left|200px]]
 {{STRUCTURE_3cki|  PDB=3cki  |  SCENE=  }}
+===Crystal structure of the TACE-N-TIMP-3 complex===
+{{ABSTRACT_PUBMED_18638486}}
-===Crystal structure of the TACE-N-TIMP-3 complex===
+==Disease==
+[[http://www.uniprot.org/uniprot/ADA17_HUMAN ADA17_HUMAN]] Defects in ADAM17 are a cause of neonatal inflammatory skin and bowel disease (NISBD) [MIM:[http://omim.org/entry/614328 614328]]. NISBD is a disorder characterized by inflammatory features with neonatal onset, involving the skin, hair, and gut. The skin lesions involve perioral and perianal erythema, psoriasiform erythroderma, with flares of erythema, scaling, and widespread pustules. Gastrointestinal symptoms include malabsorptive diarrhea that is exacerbated by intercurrent gastrointestinal infections. The hair is short or broken, and the eyelashes and eyebrows are wiry and disorganized.<ref>PMID:22010916</ref> [[http://www.uniprot.org/uniprot/TIMP3_HUMAN TIMP3_HUMAN]] Defects in TIMP3 are the cause of Sorsby fundus dystrophy (SFD) [MIM:[http://omim.org/entry/136900 136900]]. SFD is a rare autosomal dominant macular disorder with an age of onset in the fourth decade. It is characterized by loss of central vision from subretinal neovascularization and atrophy of the ocular tissues. Generally, macular disciform degeneration develops in the patients eye within 6 months to 6 years.<ref>PMID:7894485</ref><ref>PMID:8634721</ref><ref>PMID:7550309</ref><ref>PMID:8728699</ref><ref>PMID:8981947</ref>
-{{ABSTRACT_PUBMED_18638486}}
+==Function==
+[[http://www.uniprot.org/uniprot/ADA17_HUMAN ADA17_HUMAN]] Cleaves the membrane-bound precursor of TNF-alpha to its mature soluble form. Responsible for the proteolytical release of soluble JAM3 from endothelial cells surface. Responsible for the proteolytic release of several other cell-surface proteins, including p75 TNF-receptor, interleukin 1 receptor type II, p55 TNF-receptor, transforming growth factor-alpha, L-selectin, growth hormone receptor, MUC1 and the amyloid precursor protein. Also involved in the activation of Notch pathway (By similarity).<ref>PMID:12441351</ref><ref>PMID:20592283</ref> [[http://www.uniprot.org/uniprot/TIMP3_HUMAN TIMP3_HUMAN]] Complexes with metalloproteinases (such as collagenases) and irreversibly inactivates them by binding to their catalytic zinc cofactor. May form part of a tissue-specific acute response to remodeling stimuli. Known to act on MMP-1, MMP-2, MMP-3, MMP-7, MMP-9, MMP-13, MMP-14 and MMP-15.
 ==About this Structure==
@@ Line 14: / Line 16: @@
 ==Reference==
-<ref group="xtra">PMID:018638486</ref><references group="xtra"/>
+<ref group="xtra">PMID:018638486</ref><references group="xtra"/><references/>
 [[Category: ADAM 17 endopeptidase]]
 [[Category: Homo sapiens]]