Mouse anti-Human, Mouse UNC13D Monoclonal Antibody | anti-UNC13D antibody

UNC13D (Protein Unc-13 Homolog D, Munc13-4) (Biotin)

Cat. Num. AAA6145073

• Gene Names: UNC13D; FHL3; HLH3; HPLH3; Munc13-4
• Reactivity: Human, Mouse
• Applications: ELISA, Western Blot
• Purity: Purified by Protein A Affinity Chromatography.
• Synonyms: UNC13D; Monoclonal Antibody; UNC13D (Protein Unc-13 Homolog D; Munc13-4) (Biotin); anti-UNC13D antibody

• Host: Mouse
• Reactivity: Human, Mouse
• Clonality: Monoclonal
• Isotype: IgG2a,k
• Clone Number: 2C7
• Specificity: Recognizes human UNC13D. Species Crossreactivity: mouse.
• Purity/Purification: Purified by Protein A Affinity Chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Biotin.

• Applicable Applications for anti-UNC13D antibody: ELISA (EIA), Western Blot (WB)
• Application Notes: Applications are based on unconjugated antibody.
• Immunogen: Partial recombinant protein corresponding to aa2-100 from human UNC13D (NP_954712) with GST tag. MW of the GST tag alone is 26kD.
• Immunogen Sequence: ATLLSHPQQRPPFLRQAIKIRRRRVRDLQDPPPQMAPEIQPPSHHFSPEQRALLYEDALYTVLHRLGHPEPNHVTEASELLRYLQEAFHVEPEEHQQTL
• Conjugate: Biotin
• Preparation and Storage: Store product at 4 degree C if to be used immediately within two weeks. For long-term storage, aliquot to avoid repeated freezing and thawing and store at -20 degree C. Aliquots are stable at -20 degree C for 12 months after receipt. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Western Blot (WB)

(Western Blot detection against Immunogen (37kD).)

Western Blot (WB)

(UNC13D monoclonal antibody. Western Blot analysis of UNC13D expression in Jurkat.)

Western Blot (WB)

(UNC13D monoclonal antibody. Western Blot analysis of UNC13D expression in NIH/3T3.)

Testing Data

(Detection limit for recombinant GST tagged UNC13D is 0.3ng/ml as a capture antibody.)

Related Product Information for anti-UNC13D antibody

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.

Product Categories/Family for anti-UNC13D antibody

Antibodies; Proteins

NCBI and Uniprot Product Information

• NCBI GI #: 46195765
• NCBI GeneID: 201294
• NCBI Accession #: NP_954712.1
• NCBI GenBank Nucleotide #: NM_199242
• UniProt Accession #: Q70J99; Q9H7K5; B4DWG9
• Molecular Weight: 128,819 Da
• NCBI Official Full Name: protein unc-13 homolog D
• NCBI Official Synonym Full Names: unc-13 homolog D (C. elegans)
• NCBI Official Symbol: UNC13D
• NCBI Official Synonym Symbols: FHL3; HLH3; HPLH3; Munc13-4
• NCBI Protein Information: protein unc-13 homolog D
• UniProt Protein Name: Protein unc-13 homolog D
• Protein Family: Protein
• UniProt Gene Name: UNC13D
• UniProt Entry Name: UN13D_HUMAN

NCBI Description

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]

Uniprot Description

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: recycling endosome; membrane; lysosome; late endosome

Molecular Function: protein binding; Rab GTPase binding

Biological Process: regulation of mast cell degranulation; natural killer cell degranulation; germinal center formation; defense response to virus; granuloma formation; phagocytosis; positive regulation of exocytosis

Disease: Hemophagocytic Lymphohistiocytosis, Familial, 3