4leo
From Proteopedia
Contents |
Crystal structure of anti-HER3 Fab RG7116 in complex with the extracellular domains of human Her3 (ERBB3)
Template:ABSTRACT PUBMED 23780344
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
4leo is a 3 chain structure with sequence from Homo sapiens and Mus musculus. Full crystallographic information is available from OCA.
Reference
- Mirschberger C, Schiller CB, Schraml M, Dimoudis N, Friess T, Gerdes CA, Reiff U, Lifke V, Hoelzlwimmer G, Kolm I, Hopfner KP, Niederfellner G, Bossenmaier B. RG7116, a therapeutic antibody that binds the inactive HER3 receptor and is optimized for immune effector activation. Cancer Res. 2013 Jun 18. PMID:23780344 doi:10.1158/0008-5472.CAN-13-0099
- ↑ 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
- ↑ 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
