HCLS1 Associated Protein X-1 (HAX1) (AA 76-175) antibody

Details for Product No. ABIN393488
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Antigen
Synonyms HAX1, hax1, HCLSBP1, HS1BP1, SCN3, HAX-1, Hs1bp1, HSP1BP-1, SIG-111, Silg111, mHAX-1s
Epitope
AA 76-175
(17), (14), (7), (6), (5), (4), (2), (1), (1)
Reactivity
Human
(78), (20), (19), (12), (12), (11)
Host
Mouse
(44), (30), (5)
Clonality (Clone)
Monoclonal ()
Conjugate
Un-conjugated
(3), (3), (3), (1), (1), (1), (1), (1), (1), (1), (1), (1), (1), (1)
Application
Western Blotting (WB)
(61), (48), (15), (9), (5), (1), (1)
Pubmed 5 references available
Quantity 0.1 mg
Options
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Catalog No. ABIN393488
450.00 $
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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
NCBI Accession NP_006109
Research Area Phospho-specific antibodies, Protein Modifications, Cell Structure
Application Notes Western blot = 1:500-1000
Comment

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
Precaution of Use This product contains sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
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
Supplier Images
anti-HCLS1 Associated Protein X-1 (HAX1) (AA 76-175) antibody Western Blot analysis of HAX1 expression in transfected 293T cell line by HAX1 monoclonal antibody (M04), clone 1D2. Lane 1: HAX1 transfected lysate(31.6 KDa). Lane 2: Non-transfected lysate.
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).

Validation Images
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