Anti-UNC13D monoclonal antibody (DCABH-173)

Rabbit anti-Human UNC13D monoclonal antibody for WB, ICC, FC Datasheet

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Specifications


Host Species
Rabbit
Antibody Isotype
IgG
Clone
FQS5025
Species Reactivity
Mouse, Human
Immunogen
Synthetic peptide from a region of human Munc 13-4 (Q70J99).
Conjugate
Unconjugated

Applications


Application Notes
WB: 1/1000 - 1/10000; ICC: 1/250 - 1/500; Flow Cyt: 1/10 - 1/100;
*Suggested working dilutions are given as a guide only. It is recommended that the user titrates the product for use in their own experiment using appropriate negative and positive controls.

Target


Alternative Names
UNC13D; unc-13 homolog D (C. elegans); protein unc-13 homolog D; Munc13 4; FHL3; HLH3
Entrez Gene ID
UniProt ID

Product Background


Gene summary
UNC13D (Unc-13 Homolog D) is a Protein Coding gene. Diseases associated with UNC13D include hemophagocytic lymphohistiocytosis, familial, 3 and hemophagocytic lymphohistiocytosis. Among its related pathways are wtCFTR and deltaF508 traffic / Late endosome and Lysosome (norm and CF) and Deregulation of Rab and Rab Effector Genes in Bladder Cancer. GO annotations related to this gene include Rab GTPase binding. An important paralog of this gene is BAIAP3. 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.
Antigen Description
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. Familial hemophagocytic lymphohistiocytosis 3 (FHL3) [MIM:608898]: A rare disorder characterized by immune dysregulation with hypercytokinemia, defective function of natural killer cell, and massive infiltration of several organs by activated lymphocytes and macrophages. The clinical features of the disease include fever, hepatosplenomegaly, cytopenia, and less frequently neurological abnormalities ranging from irritability and hypotonia to seizures, cranial nerve deficits and ataxia. Note=The disease is caused by mutations affecting the gene represented in this entry. Protein unc-13 homolog D, also known as munc13-4, is a protein that in humans is encoded by the UNC13D gene. Munc13-4 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.

Citations


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References


Wood, SM; Meeths, M; et al. Different NK cell-activating receptors preferentially recruit Rab27a or Munc13-4 to perforin-containing granules for cytotoxicity. BLOOD 114:4117-4127(2009).
Luo, T; Kuriakose, JA; et al. Ehrlichia chaffeensis TRP120 Interacts with a Diverse Array of Eukaryotic Proteins Involved in Transcription, Signaling, and Cytoskeleton Organization. INFECTION AND IMMUNITY 79:4382-4391(2011).

Custom Antibody Labeling


We offer labeled antibodies using our catalogue antibody products and a broad range of intensely fluorescent dyes and labels including HRP, biotin, ALP, Alexa Fluor® dyes, DyLight® Fluor dyes, R-phycoerythrin (R-PE), at scales from less than 100 μg up to 1 g of IgG antibody. Learn More

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