Mouse Anti-HAX1 Recombinant Antibody (9G3D11) (CBMAB-AO548LY)

Name: Mouse Anti-HAX1 Recombinant Antibody (9G3D11)
SKU: CBMAB-AO548LY
Rating: 5 (1 reviews)

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Datasheet

SDS

Request for COA

Datasheet Target References Q & As Review & reward Protocols Associated Products

Basic Information

Host Animal

Mouse

Clone

9G3D11

Application

ELISA, WB

Immunogen

KLH-conjugated synthetic peptide encompassing a sequence within the N-term region of human Hax1a.

Specificity

Human, Rat

Antibody Isotype

IgG1

Clonality

Monoclonal

Application Notes

The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format

Liquid

Preservative

0.02% sodium azide

Concentration

1 mg/ml

Storage

Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.

More Infomation

Target

Full Name

HCLS1 Associated Protein X-1

Introduction

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]

Entrez Gene ID

Human	10456
Rat	291202

UniProt ID

Human	O00165
Rat	Q7TSE9

Alternative Names

Hax1a; HS-1 associated protein X-1; HCLS1 associated protein X-1; HCLSBP1; HS1BP1

Function

Recruits the Arp2/3 complex to the cell cortex and regulates reorganization of the cortical actin cytoskeleton via its interaction with KCNC3 and the Arp2/3 complex (PubMed:26997484).

Slows down the rate of inactivation of KCNC3 channels (PubMed:26997484).

Promotes 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. Promotes cell survival. May regulate intracellular calcium pools.

Biological Process

Cellular response to cytokine stimulus Source: BHF-UCL
Negative regulation of apoptotic process Source: UniProtKB
Positive regulation of actin cytoskeleton reorganization Source: BHF-UCL
Positive regulation of granulocyte differentiation Source: BHF-UCL
Positive regulation of peptidyl-serine phosphorylation Source: BHF-UCL
Positive regulation of peptidyl-tyrosine phosphorylation Source: BHF-UCL
Positive regulation of phosphatidylinositol 3-kinase signaling Source: BHF-UCL
Positive regulation of protein kinase B signaling Source: BHF-UCL
Positive regulation of transcription by RNA polymerase II Source: BHF-UCL
Regulation of actin filament polymerization Source: BHF-UCL
Regulation of apoptotic process Source: ParkinsonsUK-UCL
Regulation of autophagy of mitochondrion Source: ParkinsonsUK-UCL
Regulation of protein targeting to mitochondrion Source: ParkinsonsUK-UCL

Cellular Location

Mitochondrion; Cell membrane; Endoplasmic reticulum; Nucleus membrane; Cytoplasmic vesicle; Cell cortex; Sarcoplasmic reticulum; P-body
Isoform 1: Cytoplasm; Nucleus. Predominantly cytoplasmic. Also detected in the nucleus when nuclear export is inhibited, and in response to cellular stress caused by arsenite (in vitro).
Isoform 3: Cytoplasm; Nucleus. Predominantly cytoplasmic. Also detected in the nucleus when nuclear export is inhibited (in vitro).
Isoform 4: Cytoplasm; Nucleus. Shuttles between nucleus and cytoplasm.
Isoform 5: Cytoplasm. Predominantly cytoplasmic.

Involvement in disease

Neutropenia, severe congenital 3, autosomal recessive (SCN3):
The disease is caused by variants affecting the gene represented in this entry. 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 (PubMed:18337561). 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.

PTM

Proteolytically cleaved by caspase-3 during apoptosis.

Wu, Z. Y., Wang, Y., Hu, H., Ai, X. N., Zhang, Q., & Qin, Y. G. (2023). Long Noncoding RNA Cytoskeleton Regulator RNA Suppresses Apoptosis in Hepatoma Cells by Modulating the miR-125a-5p/HS1-Associated Protein X-1 Axis to Induce Caspase-9 Inactivation. Gut and Liver.

Quatrana, A., Morini, E., Tiano, F., Vancheri, C., Panarello, L., Romano, S., ... & Malisan, F. (2022). Hsa-miR223-3p circulating level is upregulated in Friedreich’s ataxia and inversely associated with HCLS1 associated protein X-1, HAX-1. Human Molecular Genetics, 31(12), 2010-2022.

Fan, Y., Murgia, M., Linder, M. I., Mizoguchi, Y., Wang, C., Łyszkiewicz, M., ... & Klein, C. (2022). HAX1-dependent control of mitochondrial proteostasis governs neutrophil granulocyte differentiation. The Journal of Clinical Investigation, 132(9).

Lin, J., Wang, Y., & Lin, Z. (2021). HAX1 maintains the glioma progression in hypoxia through promoting mitochondrial fission. Journal of Cellular and Molecular Medicine, 25(24), 11170-11184.

Choi, H. Y., Park, N., Lee, B., Choe, Y. I., Woo, D. K., Park, J. Y., & Yoo, J. C. (2020). CPNE1-mediated neuronal differentiation can be inhibited by HAX1 expression in HiB5 cells. Biochemical and biophysical research communications, 533(3), 319-324.

Wakula, M., Balcerak, A., Rubel, T., Chmielarczyk, M., Konopinski, R., Lyczek, F., & Grzybowska, E. A. (2020). The interactome of multifunctional HAX1 protein suggests its role in the regulation of energy metabolism, de-aggregation, cytoskeleton organization and RNA-processing. Bioscience Reports, 40(11), BSR20203094.

Wu, Z., Ai, X., Hu, H., Wang, S., Wang, Y., Kang, F., ... & Zhu, J. (2019). Hematopoietic-substrate-1 associated protein X-1 (HAX-1) regulates liver cancer cells growth, metastasis, and angiogenesis through Akt. Cancer Biology & Therapy, 20(9), 1223-1233.

Lu, X., Xue, P., Fu, L., Zhang, J., Jiang, J., Guo, X., ... & Cui, Z. (2018). HAX1 is associated with neuronal apoptosis and astrocyte proliferation after spinal cord injury. Tissue and Cell, 54, 1-9.

Li, Y. L., Cai, W. F., Wang, L., Liu, G. S., Paul, C., Jiang, L., ... & Wu, S. Z. (2018). Identification of the functional autophagy-regulatory domain in HCLS1-associated protein X-1 that resists against oxidative stress. DNA and Cell Biology, 37(5), 432-441.

Li, X., Qu, B., He, G., Cardona, C. J., Song, Y., & Xing, Z. (2018). Critical role of HAX-1 in promoting avian influenza virus replication in lung epithelial cells. Mediators of inflammation, 2018.

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Please try the standard protocols which include: protocols, troubleshooting and guide.

Enzyme-linked Immunosorbent Assay (ELISA)
Flow Cytometry
Immunofluorescence (IF)
Immunohistochemistry (IHC)
Immunoprecipitation (IP)
Western Blot (WB)
Enzyme Linked Immunospot (ELISpot)
Proteogenomic
Other Protocols

Alternative Versions

Mouse Anti-HAX1 (N-terminus) Recombinant Antibody (9G3D11) (CAT#: CBMAB-H1613-FY)

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For research use only. Not intended for any clinical use.

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