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GeneBio Systems

Anti-HAX1 Antibody Picoband®

Anti-HAX1 Antibody Picoband®

SKU:A01495-2

Regular price ¥94,200 JPY
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Size: 100 μg

Storage: At -20℃ for one year from date of receipt. After reconstitution, at 4℃ for one month. It can also be aliquotted and stored frozen at -20℃ for six months. Avoid repeated freezing and thawing.

Form: Lyophilized

Reactivity: Human

Applications: WB,IHC,Flow Cytometry,ELISA

Application Details: Western blot, 0.25-0.5 μg/ml, Human
Immunohistochemistry(Paraffin-embedded Section), 2-5 μg/ml, Human
Flow Cytometry (Fixed), 1-3 μg/1x10^6 cells, Human
ELISA, 0.1-0.5 μg/ml, -

Gene Name: HAX1

Specificity:

Background: HCLS1-associated protein X-1 is a protein that in humans is encoded by the HAX1 gene. 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.

Immunogen: E.coli-derived human HAX1 recombinant protein (Position: M1-R279).

Clonality: Polyclonal

Contents: Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4.

Purification: Immunogen affinity purified.

Reconstitution: Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Reference: 1. Carlsson, G., Fasth, A. Infantile genetic agranulocytosis, morbus Kostmann: presentation of six cases from the original 'Kostmann family' and a review. Acta Paediat. 90: 757-764, 2001. 2. Chao, J.-R., Parganas, E., Boyd, K., Hong, C. Y., Opferman, J. T., Ihle, J. N. Hax1-mediated processing of HtrA2 by Parl allows survival of lymphocytes and neurons. Nature 452: 98-102, 2008. Note: Erratum: Nature 452: 900 only, 2008. 3. Dale, D. C., Person, R. E., Bolyard, A. A., Aprikyan, A. G., Bos, C., Bonilla, M. A., Boxer, L. A., Kannourakis, G., Zeidler, C., Welte, K., Benson, K. F., Horwitz, M. Mutations in the gene encoding neutrophil elastase in congenital and cyclic neutropenia. Blood 96: 2317-2322, 2000.

Uniprot ID: O00165

Host: Rabbit

Concentration: Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Conjugate:

Cross Reactivity: No cross-reactivity with other proteins.

Isotype: Rabbit IgG

Phospho_site:

Clone Number:

Observed Molecular Weight: 36 kDa

Calculated Molecular Weight: 75679 MW

Gene ID: 10456

Protein Name: HCLS1-associated protein X-1

Gene Full Name: HCLS1 associated protein X-1

Synonyms: HAX 1; HAX1; HCLS1 associated protein X 1; HCLSBP1; HS1 associating protein X 1; HS1 binding protein 1; HS1BP1; HSP1BP 1; SCN3

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