4otw

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Template:STRUCTURE 4otw

Contents

HER3 pseudokinase domain bound to bosutinib

Template:ABSTRACT PUBMED 24656791

Disease

[ERBB3_HUMAN] Defects in ERBB3 are the cause of lethal congenital contracture syndrome type 2 (LCCS2) [MIM:607598]; also called Israeli Bedouin multiple contracture syndrome type A. LCCS2 is an autosomal recessive neurogenic form of a neonatally lethal arthrogryposis that is associated with atrophy of the anterior horn of the spinal cord. The LCCS2 syndrome is characterized by multiple joint contractures, anterior horn atrophy in the spinal cord, and a unique feature of a markedly distended urinary bladder. The phenotype suggests a spinal cord neuropathic etiology.[1]

Function

[ERBB3_HUMAN] Binds and is activated by neuregulins and NTAK.[2]

About this Structure

4otw is a 1 chain structure. Full crystallographic information is available from OCA.

Reference

  • Littlefield P, Moasser MM, Jura N. An ATP-Competitive Inhibitor Modulates the Allosteric Function of the HER3 Pseudokinase. Chem Biol. 2014 Mar 18. pii: S1074-5521(14)00069-6. doi:, 10.1016/j.chembiol.2014.02.011. PMID:24656791 doi:http://dx.doi.org/10.1016/j.chembiol.2014.02.011
  1. ↑ Narkis G, Ofir R, Manor E, Landau D, Elbedour K, Birk OS. Lethal congenital contractural syndrome type 2 (LCCS2) is caused by a mutation in ERBB3 (Her3), a modulator of the phosphatidylinositol-3-kinase/Akt pathway. Am J Hum Genet. 2007 Sep;81(3):589-95. Epub 2007 Jul 24. PMID:17701904 doi:S0002-9297(07)61355-X
  2. ↑ Kinugasa Y, Ishiguro H, Tokita Y, Oohira A, Ohmoto H, Higashiyama S. Neuroglycan C, a novel member of the neuregulin family. Biochem Biophys Res Commun. 2004 Sep 3;321(4):1045-9. PMID:15358134 doi:10.1016/j.bbrc.2004.07.066

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