ZEB2 | SIP1 Rabbit pAb (一抗) | Bioss

bs-20484R

宠物抗体、农牧业/家禽抗体

ZEB2 | SIP1 Rabbit pAb

Smad蛋白相互作用蛋白1抗体

HSPC082; SIP-1; SIP1; SMADIP1; ZFHX1B; 9130203F04Rik; D130016B08Rik; Zfx1b; Zfxh1b; ZEB2_HUMAN; ZEB2; Smad-interacting protein 1 (SMADIP1); Zinc finger homeobox protein 1b; KIAA0569; ZEB2_MOUSE; Smad-interacting protein 1; zinc finger E-box binding homeobox 2; zinc finger homeobox 1b; SMAD interacting protein 1

Rabbit

KLH conjugated synthetic peptide derived from human SIP1: 1061-1160/1214

IgG

Liquid

affinity purified by Protein A

Polyclonal

136 kDa

133 kDa

1mg/ml

0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.

Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.

This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

SMAD regulates gene expression by interacting with different classes of transcription factors including DNA-binding multi-zinc finger proteins. SIP1, for SMAD interacting protein 1, is a member of the delta-EF1/Zfh1 family of 2-handed zinc finger/homeodomain proteins. SIP1 contains a SMAD-binding domain, a homeodomain and two clusters of zinc fingers on the N- and C-termini. SIP1, also known as SMADIP1, ZFHX1B and ZEB2 (zinc finger E-box-binding protein 2), can be induced by TGF∫ treatment. SIP1 plays a crucial role in normal embryonic development of neural structures and the neural crest. The human SIP1 gene maps to chromosome 2q22. Mutations in the SIP1 gene cause a form of Hirschsprung disease (HSCR). Patients with SIP1 mutations show mental retardation, delayed motor development, epilepsy, microcephaly, distinct facial features and/or congenital heart disease—all symptoms of HSCR.

ZEB2

Zinc finger E-box-binding homeobox 2

Binds activated SMAD1, activated SMAD2 and activated SMAD3; binding with SMAD4 is not detected (By similarity). Interacts with CBX4 and CTBP1.

Nucleus.

Sumoylation on Lys-391 and Lys-866 is promoted by the E3 SUMO-protein ligase CBX4, and impairs interaction with CTBP1 and transcription repression activity.

Defects in ZEB2 are the cause of Mowat-Wilson syndrome (MWIS) [MIM:235730]; also known as Hirschsprung disease-mental retardation syndrome. A complex developmental disorder characterized by mental retardation, delayed motor development, epilepsy, microcephaly and a wide spectrum of clinically heterogeneous features suggestive of neurocristopathies at the cephalic, cardiac, and vagal levels. Some patients manifest Hirschsprung disease. Affected patients show an easily recognizable facial appearance with deep set eyes and hypertelorism, medially divergent, broad eyebrows, prominent columella, pointed chin and uplifted, notched ear lobes.

Belongs to the delta-EF1/ZFH-1 C2H2-type zinc-finger family.
Contains 7 C2H2-type zinc fingers.
Contains 1 homeobox DNA-binding domain.

Transcriptional inhibitor that binds to DNA sequence 5'-CACCT-3' in different promoters. Represses transcription of E-cadherin.