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MGP003186

UniProt Annotations

Entry Information
Gene Namethyroid peroxidase
Protein EntryPERT_HUMAN
UniProt IDP07202
SpeciesHuman
Comments
Comment typeDescription
Alternative ProductsEvent=Alternative splicing; Named isoforms=8; Comment=Additional isoforms seem to exist.; Name=1; Synonyms=TPO1; IsoId=P07202-1; Sequence=Displayed; Name=2; Synonyms=TPO2; IsoId=P07202-2; Sequence=VSP_004665; Note=Lacks exon 10. Found in normal thyroid tissues as well as Graves'tissues. Rapidly degraded after synthesis, does not reach the cell surface. Inactive.; Name=3; Synonyms=TPO3, Graves' disease, TPOzaninelli; IsoId=P07202-3; Sequence=VSP_004666; Note=Lacks exon 16. Found in normal thyroid tissues as well as Graves'tissues. Active.; Name=4; Synonyms=TPO4; IsoId=P07202-4; Sequence=VSP_007269; Note=Lacks exon 14. Active.; Name=5; Synonyms=TPO5; IsoId=P07202-5; Sequence=VSP_007268; Note=Lacks exon 8. Does not fold correctly. Does not reach the cell surface.; Name=6; Synonyms=TPO6; IsoId=P07202-6; Sequence=VSP_004665, VSP_007270; Note=Lacks exons 10, 12, 13, 14 and 16.; Name=2-3; IsoId=P07202-7; Sequence=VSP_004665, VSP_004666; Note=Lacks exons 10 and 16.; Name=2-4; IsoId=P07202-8; Sequence=VSP_004665, VSP_007269; Note=Lacks exons 10 and 14.;
Catalytic Activity2 iodide + H(2)O(2) + 2 H(+) = 2 iodine + 2 H(2)O.
Catalytic Activity2 [thyroglobulin]-3,5-diiodo-L-tyrosine + H(2)O(2) = [thyroglobulin]-L-thyroxine + [thyroglobulin]- aminoacrylate + 2 H(2)O.
Catalytic Activity[Thyroglobulin]-3-iodo-L-tyrosine + iodide + H(2)O(2) = [thyroglobulin]-3,5-diiodo-L-tyrosine + 2 H(2)O.
Catalytic Activity[Thyroglobulin]-3-iodo-L-tyrosine + [thyroglobulin]-3,5-diiodo-L-tyrosine + H(2)O(2) = [thyroglobulin]-3,5,3'-triiodo-L-thyronine + [thyroglobulin]- aminoacrylate + 2 H(2)O.
Catalytic Activity[Thyroglobulin]-L-tyrosine + iodide + H(2)O(2) = [thyroglobulin]-3-iodo-L-tyrosine + 2 H(2)O.
CofactorName=Ca(2+); Xref=ChEBI:CHEBI:29108; Evidence={ECO:0000255|PROSITE-ProRule:PRU00298}; Note=Binds 1 Ca(2+) ion per heterodimer. {ECO:0000255|PROSITE- ProRule:PRU00298};
CofactorName=heme b; Xref=ChEBI:CHEBI:60344; Evidence={ECO:0000255|PROSITE-ProRule:PRU00298}; Note=Binds 1 heme b (iron(II)-protoporphyrin IX) group covalently per heterodimer. {ECO:0000255|PROSITE-ProRule:PRU00298};
DiseaseNote=An alternative splicing in the thyroperoxidase mRNA can cause Graves' disease.
DiseaseThyroid dyshormonogenesis 2A (TDH2A) [MIM:274500]: A disorder due to defective conversion of accumulated iodide to organically bound iodine. The iodide organification defect can be partial or complete. {ECO:0000269|PubMed:10084596, ECO:0000269|PubMed:10468986, ECO:0000269|PubMed:11061528, ECO:0000269|PubMed:11415848, ECO:0000269|PubMed:11874711, ECO:0000269|PubMed:11916616, ECO:0000269|PubMed:12213873, ECO:0000269|PubMed:12490071, ECO:0000269|PubMed:12843174, ECO:0000269|PubMed:12864797, ECO:0000269|PubMed:12938097, ECO:0000269|PubMed:16284446, ECO:0000269|PubMed:16684826, ECO:0000269|PubMed:7550241, ECO:0000269|PubMed:9024270, ECO:0000269|PubMed:9924196}. Note=The disease is caused by mutations affecting the gene represented in this entry.
FunctionIodination and coupling of the hormonogenic tyrosines in thyroglobulin to yield the thyroid hormones T(3) and T(4).
PathwayHormone biosynthesis; thyroid hormone biosynthesis.
PtmCleaved in its N-terminal part.
PtmGlycosylated.
PtmHeme is covalently bound through a H(2)O(2)-dependent autocatalytic process. Heme insertion is important for the delivery of protein at the cell surface.
SimilarityBelongs to the peroxidase family. XPO subfamily. {ECO:0000255|PROSITE-ProRule:PRU00298}.
SimilarityContains 1 EGF-like domain. {ECO:0000255|PROSITE- ProRule:PRU00076}.
SimilarityContains 1 Sushi (CCP/SCR) domain. {ECO:0000255|PROSITE-ProRule:PRU00302}.
Subcellular LocationIsoform 3: Cell surface.
Subcellular LocationMembrane; Single-pass type I membrane protein.
SubunitInteracts with DUOX1, DUOX2 and CYBA. {ECO:0000269|PubMed:15561711}.
Web ResourceName=Wikipedia; Note=Thyroid peroxidase entry; URL="http://en.wikipedia.org/wiki/Thyroid_peroxidase";
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