Product Name	Anti-HAX1/AF594
Chinese Name	AF594标记的造血Stem cells特异性相关Binding protein1抗体
Alias	HAX 1; Hax1a; HCLS1 and PKD2 associated protein; HCLS1 associated protein; HCLS1 associated protein X 1; HCLSBP1; HS 1 associated protein X 1; HS 1 binding protein; HS1 associating protein X 1; HS1 binding protein 1; HS1 binding protein; HS1BP1; SCN3.
Research Area	Cell biology  Apoptosis  Epigenetics
Immunogen Species	Rabbit
Clonality	Polyclonal
React Species
Applications	ICC=1:50-200 IF=1:50-200  not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
Molecular weight	31kDa
Form	Lyophilized or Liquid
Concentration	1mg/ml
immunogen	KLH conjugated synthetic peptide derived from human HAX1
Lsotype	IgG
Purification	affinity purified by Protein A
Storage Buffer	0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Storage	Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
Product Detail	background: RelevanceHAX1 associates with HS1, binding to its N-terminal region. It is also known to associate with PKD2 (involved in polycystic kidney disease) and with cortactin/EMS1. HAX1 is also reported to bind to hairpin structures in vimentin and DNA polymerase beta mRNAs, so may play a role in mRNA stability and transport. It may also function in promoting cell survival. Defects in HAX1 are the cause of autosomal recessive severe congenital neutropenia 3 (SCN3) also called Kostmann disease. Function: 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. Subunit: Interacts with ABCB1, ABCB4 and ABCB11 (By similarity). Directly associates with HCLS1/HS1, through binding to its N-terminal region. Interacts with CTTN. Interacts with PKD2. Interacts with GNA13. Interacts with CASP9. Interacts with ITGB6. Interacts with PLN and ATP2A2; these interactions are inhibited by calcium. Interacts with GRB7. Interacts (via C-terminus) with XIAP/BIRC4 (via BIR 2 domain and BIR 3 domain) and this interaction blocks ubiquitination of XIAP/BIRC4. Subcellular Location: Mitochondrion. Endoplasmic reticulum. Nucleus membrane. Cytoplasmic vesicle (By similarity). Sarcoplasmic reticulum (By similarity). Tissue Specificity: Ubiquitous. Up-regulated in oral cancers. Post-translational modifications: Proteolytically cleaved by caspase-3 during apoptosis. DISEASE: Defects in HAX1 are the cause of neutropenia severe congenital autosomal recessive type 3 (SCN3) [MIM:610738]; 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. Note=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. Similarity: Belongs to the HAX1 family. Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. HAX1蛋白在Mitochondrion蛋白广泛表达，是一种抗凋亡蛋白，与bel-2有相似的机制。

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