SEDL Antibody (JK-4): sc-101312

SEDL Antibody (JK-4) is a mouse monoclonal IgG2b kappa light chain antibody that detects SEDL protein of mouse, rat, and human origin by western blotting (WB), immunoprecipitation (IP), and enzyme-linked immunosorbent assay (ELISA). Anti-SEDL antibody (JK-4) is available as the non-conjugated form. SEDL, also known as TRAPPC2 (trafficking protein particle complex subunit 2), SEDT, TRS20, MIP-2A (MBP-1-interacting protein 2A), or ZNF547L, is an evolutionarily conserved member of the Sedlin subfamily within the TRAPP small subunits family of proteins. SEDL protein is primarily located in the perinuclear region of the cytoplasm, where SEDL plays a crucial role in cellular transport processes. By binding to α Enolase, SEDL protein sequesters α Enolase in the cytoplasm, thereby inhibiting transcriptional repression and preventing α Enolase-mediated cell death, which is vital for maintaining cellular homeostasis. Furthermore, SEDL protein is integral to the transport mechanism from the endoplasmic reticulum to the Golgi apparatus, functioning as a key component of the multisubunit transport protein particle (TRAPP) complex. Mutations in the gene encoding SEDL can lead to late childhood onset of spondyloepiphyseal dysplasia tarda (SEDT), an X-linked recessive disorder that affects endochondral bone formation, particularly in males, resulting in a short trunk and hips along with degenerative changes.

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SEDL Antibody (JK-4) References:

1. Interaction of Sedlin with chloride intracellular channel proteins.  |  Fan, L., et al. 2003. FEBS Lett. 540: 77-80. PMID: 12681486
2. Identification of three novel SEDL mutations, including mutation in the rare, non-canonical splice site of exon 4.  |  Shaw, MA., et al. 2003. Clin Genet. 64: 235-42. PMID: 12919139
3. Spondyloepiphyseal dysplasia tarda (SEDL, MIM #313400).  |  Savarirayan, R., et al. 2003. Eur J Hum Genet. 11: 639-42. PMID: 12939648
4. Human wild-type SEDL protein functionally complements yeast Trs20p but some naturally occurring SEDL mutants do not.  |  Gécz, J., et al. 2003. Gene. 320: 137-44. PMID: 14597397
5. [Gene diagnosis of X-linked spondyloepiphyseal dysplasia tarda by linkage analysis and DNA sequencing].  |  Wang, HL., et al. 2003. Zhonghua Er Ke Za Zhi. 41: 256-9. PMID: 14754526
6. X-linked spondyloepiphyseal dysplasia tarda: a novel SEDL mutation in a Jewish Ashkenazi family and clinical intervention considerations.  |  Bar-Yosef, U., et al. 2004. Am J Med Genet A. 125A: 45-8. PMID: 14755465
7. Mutation of acceptor splice site of the SEDL gene in X-linked spondyloepiphyseal dysplasia tarda causes the activation of cryptic splice site.  |  Ma, HW., et al. 2005. Zhonghua Yi Xue Yi Chuan Xue Za Zhi. 22: 251-3. PMID: 15952107
8. The structure of the TRAPP subunit TPC6 suggests a model for a TRAPP subcomplex.  |  Kümmel, D., et al. 2005. EMBO Rep. 6: 787-93. PMID: 16025134