抗体

山羊抗Munc13-4/UNC13D(C末端)抗体

MBS420652

0.1毫克

300美元

多克隆

山羊

未共轭

免疫印迹, 酶免疫法

抗体

山羊抗Munc13-4/UNC13D(C末端)抗体

Munc13-4/UNC13D

Goat Anti-Munc13-4 / UNC13D (Internal) Antibody; UNC13D; Munc13-4; unc-13 homolog D (C. elegans); FHL3; HLH3; HPLH3; unc-13 homolog D; UNC13D antibody; Munc13-4 antibody; unc-13 homolog D (C. elegans) antibody; FHL3 antibody; HLH3 antibody; HPLH3 antibody; unc-13 homolog D antibody; Munc13-4; Munc13-4 / UNC13D (C terminus)

protein unc-13 homolog D; Protein unc-13 homolog D; protein unc-13 homolog D; unc-13 homolog D; Munc13-4

UNC13D

UNC13D;UNC13D;FHL3;HLH3;HPLH3;Munc13-4

UN13D_HUMAN

多克隆

山羊

Tested: Human; Expected from sequence similarity: Human

1090

KQASQHALRPAP

通过硫酸铵沉淀和用免疫多肽抗原亲和层析从山羊血清中纯化得到的

Supplied at 0.5 mg/ml in Tris saline, 0. 02% sodium azide, pH7.3 with 0.5% bovine serum albumin.

100ug specific antibody in 200ul

Aliquot and store at -20 degree C. Minimize freezing and thawing.

肽酶联免疫吸附测定(EIA), 免疫印迹(免疫印迹)

Peptide ELISA: Antibody detection limit dilution 1: 16000. Western Blot: Approx.105kDa band observed in Human T-lymphocyte and HeLa lysates (calculated MW of 123kDa according to NP_954712.1). Recommended concentration: 1-3ug/ml.

Immunogen: Peptide with sequence C-KQASQHALRPAP, from the C Terminus of the protein sequence according to NP_954712.1. Epitope: C Terminus

46195765

NP_954712.1

NM_199242.2

128,819 Da

Deregulation Of Rab And Rab Effector Genes In Bladder Cancer Pathway (698757)

This gene encodes a protein that is a member of the UNC13 family, containing similar domain structure as other family members but lacking an N-terminal phorbol ester-binding C1 domain present in other Munc13 proteins. The protein appears to play a role in vesicle maturation during exocytosis and is involved in regulation of cytolytic granules secretion. Mutations in this gene are associated with familial hemophagocytic lymphohistiocytosis type 3, a genetically heterogeneous, rare autosomal recessive disorder. [provided by RefSeq, Jul 2008]

UNC13D: Plays a role in cytotoxic granule exocytosis in lymphocytes. Required for both granule maturation and granule docking and priming at the immunologic synapse. Regulates assembly of recycling and late endosomal structures, leading to the formation of an endosomal exocytic compartment that fuses with perforin-containing granules at the immunologic synapse and licences them for exocytosis. Regulates Ca(2+)-dependent secretory lysosome exocytosis in mast cells. Defects in UNC13D are the cause of familial hemophagocytic lymphohistiocytosis type 3 (FHL3); also known as HPLH3. Familial hemophagocytic lymphohistiocytosis (FHL) is a genetically heterogeneous, rare autosomal recessive disorder. It is characterized by immune dysregulation with hypercytokinemia and defective natural killer cell function. The clinical features of the disease include fever, hepatosplenomegaly, cytopenia, hypertriglyceridemia, hypofibrinogenemia, and neurological abnormalities ranging from irritability and hypotonia to seizures, cranial nerve deficits, and ataxia. Hemophagocytosis is a prominent feature of the disease, and a non-malignant infiltration of macrophages and activated T-lymphocytes in lymph nodes, spleen, and other organs is also found. Belongs to the unc-13 family. 3 isoforms of the human protein are produced by alternative splicing. Chromosomal Location of Human Ortholog: 17q25.1. Cellular Component: late endosome; lysosome; membrane; recycling endosome. Molecular Function: protein binding; Rab GTPase binding. Biological Process: defense response to virus; germinal center formation; granuloma formation; natural killer cell degranulation; phagocytosis; positive regulation of exocytosis; regulation of mast cell degranulation. Disease: Hemophagocytic Lymphohistiocytosis, Familial, 3

0.1毫克

300美元