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Details for Product No. ABIN393488

HCLS1 Associated Protein X-1 (HAX1) antibody

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Synonyms HCLSBP1, HS1BP1, SCN3, HAX-1, Hs1bp1, HSP1BP-1, SIG-111, Silg111, mHAX-1s, HAX1, hax1
»Alternatives Human
»Alternatives Mouse
Clonality (Clone) Monoclonal ()
»Alternatives Un-conjugated
»Alternatives ELISA, Western Blotting (WB)
Pubmed 5 references available
Catalog no. ABIN393488
Quantity 0.1 mg
450.00 $   Plus shipping costs $45.00
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Immunogen HAX1 (NP_006109, 76 a.a. ~ 175 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 K
Clone 1D2
Isotype IgG2a kappa
Specificity HAX1 (NP_006109, 76 a.a. ~ 175 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.
Purification This antibody is purified through a protein G column, eluted with high and low pH buffers and neutralized immediately, followed by dialysis against PBS.
Alternative Name HAX1
Background Synonyms: Serine protease inhibitor J6, 47 kDa heat shock protein, Collagen-binding protein, Colligin, Serpin H1,Hsp47, Cbp1, Serpinh1
Molecular Weight 31621 DA
Gene ID 12406
UniProt NP_006109
Research Area Phospho-specific antibodies, Protein Modifications, Cell Structure
Application Notes ELISA ~~ 1ug/ml~3ng/ml Western blot ~~ 1:500~1000

Background: 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.

Restrictions For Research Use only
Buffer PBS with 0.09% (w/v) sodium azide.
Preservative Sodium azide
Storage 4 °C/-20 °C
Storage Comment Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles
Expiry Date 6 months
Background publications Faiyaz-Ul-Haque, Al-Jefri, Abalkhail et al.: "A novel missense mutation in the HAX1 gene in severe congenital neutropenia patients (Kostmann disease)." in: Clinical genetics, Vol. 76, Issue 6, pp. 569-72, 2009 (PubMed).

Ishikawa, Kobayashi: "[Neurological findings in severe congenital neutropenia with HAX1 mutations]." in: No to hattatsu. Brain and development, Vol. 41, Issue 6, pp. 415-9, 2009 (PubMed).

Kang, Jang, Park et al.: "Molecular interaction between HAX-1 and XIAP inhibits apoptosis." in: Biochemical and biophysical research communications, Vol. 393, Issue 4, pp. 794-9, 2010 (PubMed).

Han, Goldstein, Hou et al.: "Deregulation of mitochondrial membrane potential by mitochondrial insertion of granzyme B and direct Hax-1 cleavage." in: The Journal of biological chemistry, Vol. 285, Issue 29, pp. 22461-72, 2010 (PubMed).

Burnicka-Turek, Kata, Buyandelger et al.: "Pelota interacts with HAX1, EIF3G and SRPX and the resulting protein complexes are associated with the actin cytoskeleton." in: BMC cell biology, Vol. 11, pp. 28, 2010 (PubMed).

Alternatives for antigen "HCLS1 Associated Protein X-1 (HAX1)", type "Antibodies"
Hosts (37), (28), (6)
Reactivities (72), (19), (17), (12), (12), (11)
Applications (58), (48), (9), (9), (4)
Conjugates (2), (2), (2), (1), (1), (1), (1), (1), (1), (1), (1)
Epitopes (22), (8), (5), (4), (2), (2), (2), (1), (1)