1ygr: Difference between revisions

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~~[[Image:1ygr.png|left|200px]]~~

===Crystal structure of the tandem phosphatase domain of RPTP CD45===

===~~Crystal structure~~ of the ~~tandem phosphatase domain~~ of ~~RPTP CD45===~~

==Disease==

[[http://www.uniprot.org/uniprot/CD45_HUMAN CD45_HUMAN]] Defects in PTPRC are a cause of severe combined immunodeficiency autosomal recessive T-cell-negative/B-cell-positive/NK-cell-positive (T(-)B(+)NK(+) SCID) [MIM:[http://omim.org/entry/608971 608971]]. A form of severe combined immunodeficiency (SCID), a genetically and clinically heterogeneous group of rare congenital disorders characterized by impairment of both humoral and cell-mediated immunity, leukopenia, and low or absent antibody levels. Patients present in infancy recurrent, persistent infections by opportunistic organisms. The common characteristic of all types of SCID is absence of T-cell-mediated cellular immunity due to a defect in T-cell development.<ref>PMID:11145714</ref> Genetic variations in PTPRC are involved in multiple sclerosis susceptibility (MS) [MIM:[http://omim.org/entry/126200 126200]]. MS is a neurodegenerative disorder characterized by the gradual accumulation of focal plaques of demyelination particularly in the periventricular areas of the brain. Peripheral nerves are not affected. Onset usually in third or fourth decade with intermittent progression over an extended period. The cause is still uncertain.<ref>PMID:11101853</ref> [[http://www.uniprot.org/uniprot/CD3Z_HUMAN CD3Z_HUMAN]] Defects in CD247 are the cause of immunodeficiency due to defect in CD3-zeta (CD3ZID) [MIM:[http://omim.org/entry/610163 610163]]. An immunological deficiency characterized by T-cells impaired immune response to alloantigens, tetanus toxoid and mitogens.<ref>PMID:16672702</ref>

~~{{ABSTRACT_PUBMED_15684325}}~~

==Function==

[[http://www.uniprot.org/uniprot/CD45_HUMAN CD45_HUMAN]] Protein tyrosine-protein phosphatase required for T-cell activation through the antigen receptor. Acts as a positive regulator of T-cell coactivation upon binding to DPP4. The first PTPase domain has enzymatic activity, while the second one seems to affect the substrate specificity of the first one. Upon T-cell activation, recruits and dephosphorylates SKAP1 and FYN. Dephosphorylates LYN, and thereby modulates LYN activity (By similarity).<ref>PMID:2845400</ref><ref>PMID:11909961</ref> [[http://www.uniprot.org/uniprot/CD3Z_HUMAN CD3Z_HUMAN]] Probable role in assembly and expression of the TCR complex as well as signal transduction upon antigen triggering.

==About this Structure==

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==Reference==

[[Category: Homo sapiens]]

[[Category: Frederick, C A.]]

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@@ Line 1: / Line 1: @@
-[[Image:1ygr.png|left|200px]]
 {{STRUCTURE_1ygr|  PDB=1ygr  |  SCENE=  }}
+===Crystal structure of the tandem phosphatase domain of RPTP CD45===
+{{ABSTRACT_PUBMED_15684325}}
-===Crystal structure of the tandem phosphatase domain of RPTP CD45===
+==Disease==
+[[http://www.uniprot.org/uniprot/CD45_HUMAN CD45_HUMAN]] Defects in PTPRC are a cause of severe combined immunodeficiency autosomal recessive T-cell-negative/B-cell-positive/NK-cell-positive (T(-)B(+)NK(+) SCID) [MIM:[http://omim.org/entry/608971 608971]]. A form of severe combined immunodeficiency (SCID), a genetically and clinically heterogeneous group of rare congenital disorders characterized by impairment of both humoral and cell-mediated immunity, leukopenia, and low or absent antibody levels. Patients present in infancy recurrent, persistent infections by opportunistic organisms. The common characteristic of all types of SCID is absence of T-cell-mediated cellular immunity due to a defect in T-cell development.<ref>PMID:11145714</ref>  Genetic variations in PTPRC are involved in multiple sclerosis susceptibility (MS) [MIM:[http://omim.org/entry/126200 126200]]. MS is a neurodegenerative disorder characterized by the gradual accumulation of focal plaques of demyelination particularly in the periventricular areas of the brain. Peripheral nerves are not affected. Onset usually in third or fourth decade with intermittent progression over an extended period. The cause is still uncertain.<ref>PMID:11101853</ref> [[http://www.uniprot.org/uniprot/CD3Z_HUMAN CD3Z_HUMAN]] Defects in CD247 are the cause of immunodeficiency due to defect in CD3-zeta (CD3ZID) [MIM:[http://omim.org/entry/610163 610163]]. An immunological deficiency characterized by T-cells impaired immune response to alloantigens, tetanus toxoid and mitogens.<ref>PMID:16672702</ref>
-{{ABSTRACT_PUBMED_15684325}}
+==Function==
+[[http://www.uniprot.org/uniprot/CD45_HUMAN CD45_HUMAN]] Protein tyrosine-protein phosphatase required for T-cell activation through the antigen receptor. Acts as a positive regulator of T-cell coactivation upon binding to DPP4. The first PTPase domain has enzymatic activity, while the second one seems to affect the substrate specificity of the first one. Upon T-cell activation, recruits and dephosphorylates SKAP1 and FYN. Dephosphorylates LYN, and thereby modulates LYN activity (By similarity).<ref>PMID:2845400</ref><ref>PMID:11909961</ref> [[http://www.uniprot.org/uniprot/CD3Z_HUMAN CD3Z_HUMAN]] Probable role in assembly and expression of the TCR complex as well as signal transduction upon antigen triggering.
 ==About this Structure==
@@ Line 14: / Line 16: @@
 ==Reference==
-<ref group="xtra">PMID:015684325</ref><references group="xtra"/>
+<ref group="xtra">PMID:015684325</ref><references group="xtra"/><references/>
 [[Category: Homo sapiens]]
 [[Category: Frederick, C A.]]