Hax1a, Monoclonal Antibody

Hax1a Antibody [9G6C6] : SCN3; HS1BP1; HCLSBP1; HCLS1 associated protein X-1

For Research Use Only. Not for use in diagnostic procedures.

Monoclonal

IgG3

9G6C6

Mouse

Hax1a Monoclonal Antibody is immunoaffinity chromotography purified IgG.

Liquid

1 mg/mL (lot specific)

Hax1a monoclonal antibody can be stored at -20 degree C, stable for one year.

Small volumes of anti-HAX1 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Hax1a Monoclonal Antibody: The HS-1 associated protein X-1 (Hax1) was initially identified in a yeast two-hybrid assay on the basis of its ability to bind to the hemapoietic cell-specific protein 1 (HS-1). Hax1 possesses anti-apoptotic activity and is structurally related to Bcl-2 family members, including the presence of BH1- and BH2-like domains. It has recently been shown to interact with HIV viral protein R (Vpr), a protein required for viral pathogenesis of HIV and linked to T-cell apoptosis through activation of caspases 3 and 9. Other studies indicate that Hax1-mediated processing of HtrA2 (also known as Omi) by the mitochondrial protease PARL allows survival of lymphocytes and neurons when cytokines are limiting. At least four isoforms of Hax1 are known to exist. This antibody is expected to recognize the longest isoform (Hax1a) as well as the shortest.

ELISA (EIA), Western Blot (WB)

Hax1a antibody can be used for detection of Hax1A by Western blot at 1 - 2 mug/mL. For immunofluorescence start at 20 mug/mL.

Western blot analysis of Hax1a in human heart tissue lysate with Hax1a antibody at (A) 1 and (B) 2 μg/mL.

21,801 Da

HCLS1 associated protein X-1

HCLS1-associated protein X-1; HAX-1; HSP1BP-1; HS1 binding protein; HS1-binding protein 1; HS1-associating protein X-1; HCLS1 (and PKD2) associated protein

HCLS1-associated protein X-1

HS1BP1; HAX-1; HSP1BP-1  [Similar Products]

HAX1_HUMAN

The protein encoded by this gene is known to associate with hematopoietic cell-specific Lyn substrate 1, a substrate of Src family tyrosine kinases. It also interacts with the product of the polycystic kidney disease 2 gene, mutations in which are associated with autosomal-dominant polycystic kidney disease, and with the F-actin-binding protein, cortactin. It was earlier thought that this gene product is mainly localized in the mitochondria, however, recent studies indicate it to be localized in the cell body. Mutations in this gene result in autosomal recessive severe congenital neutropenia, also known as Kostmann disease. Two transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jul 2008]

HAX1: Promotes cell survival. Potentiates GNA13-mediated cell migration. Involved in the clathrin-mediated endocytosis pathway. May be involved in internalization of ABC transporters such as ABCB11. May inhibit CASP9 and CASP3. May regulate intracellular calcium pools. Defects in HAX1 are the cause of neutropenia severe congenital autosomal recessive type 3 (SCN3); also known as Kostmann disease. A disorder of hematopoiesis characterized by maturation arrest of granulopoiesis at the level of promyelocytes with peripheral blood absolute neutrophil counts below 0.5 x 10(9)/l and early onset of severe bacterial infections. Some patients affected by severe congenital neutropenia type 3 have neurological manifestations such as psychomotor retardation and seizures. The clinical phenotype due to HAX1 deficiency appears to depend on the localization of the mutations and their influence on the transcript variants. Mutations affecting exclusively isoform 1 are associated with isolated congenital neutropenia, whereas mutations affecting both isoform 1 and isoform 5 are associated with additional neurologic symptoms. Belongs to the HAX1 family. 6 isoforms of the human protein are produced by alternative splicing.

Protein type: Mitochondrial; Endoplasmic reticulum; Vesicle; Apoptosis

Chromosomal Location of Human Ortholog: 1q21.3

Cellular Component: transcription factor complex; nuclear membrane; sarcoplasmic reticulum; mitochondrion; clathrin-coated vesicle; endoplasmic reticulum; lamellipodium; apical plasma membrane; nuclear envelope; actin cytoskeleton

Molecular Function: protein domain specific binding; protein binding; protein N-terminus binding; interleukin-1 binding

Biological Process: positive regulation of phosphoinositide 3-kinase cascade; regulation of apoptosis; positive regulation of protein kinase B signaling cascade; positive regulation of peptidyl-tyrosine phosphorylation; regulation of actin filament polymerization; positive regulation of granulocyte differentiation; positive regulation of transcription from RNA polymerase II promoter; positive regulation of peptidyl-serine phosphorylation

Disease: Neutropenia, Severe Congenital, 3, Autosomal Recessive

All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.

While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.