HAX1 Antibody (PACO31704)
- SKU:
- PACO31704
- Product type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Application:
- WB
- Application:
- IHC
- Application:
- IF
- Antibody type:
- Polyclonal
- Conjugation:
- Unconjugated
Description
抗体名: | HAX1 Antibody (PACO31704) |
抗体コード: | PACO31704 |
サイズ: | 50ug |
宿主種: | Rabbit |
申し込み: | ELISA, WB, IHC, IF |
推奨される希釈: | ELISA:1:2000-1:10000, WB:1:500-1:5000, IHC:1:20-1:200, IF:1:50-1:200 |
反応性: | Human, Mouse, Rat |
免疫原: | Recombinant Human HCLS1-associated protein X-1 protein (2-250AA) |
憲法: | Liquid |
ストレージバッファ: | Preservative: 0.03% Proclin 300 Constituents: 50% Glycerol, 0.01M PBS, PH 7.4 |
精製方法: | >95%, Protein G purified |
抗体のクローン性: | Polyclonal |
アイソタイプ: | IgG |
Conjugate: | Non-conjugated |
![]() | Western Blot Positive WB detected in: Mouse brain tissue, Rat brain tissue All lanes: HAX1 antibody at 1.5µg/ml Secondary Goat polyclonal to rabbit IgG at 1/50000 dilution Predicted band size: 32, 33, 29, 15, 27, 22 kDa Observed band size: 32 kDa . |
![]() | Immunofluorescent analysis of Hela cells using PACO31704 at dilution of 1:100 and Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L). |
![]() | IHC image of PACO31704 diluted at 1:150 and staining in paraffin-embedded human gastric cancer performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system. |
バックグラウンド: | 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. Slows down the rate of inactivation of KCNC3 channels. 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. |
シノニム: | HCLS1-associated protein X-1 (HS1-associating protein X-1) (HAX-1) (HS1-binding protein 1) (HSP1BP-1), HAX1, HS1BP1 |
UniProt Protein Function: | HAX1: 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. Defects in HAX1 are the cause of neutropenia severe congenital autosomal recessive type 3 (SCN3); 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. 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. Belongs to the HAX1 family. 6 isoforms of the human protein are produced by alternative splicing. |
UniProt Protein Details: | Protein type:Endoplasmic reticulum; Apoptosis; Vesicle; Mitochondrial Chromosomal Location of Human Ortholog: 1q21.3 Cellular Component: transcription factor complex; nuclear membrane; mitochondrion; clathrin-coated vesicle; sarcoplasmic reticulum; lamellipodium; endoplasmic reticulum; apical plasma membrane; nuclear envelope; actin cytoskeleton Molecular Function:protein domain specific binding; protein binding; protein N-terminus binding; interleukin-1 binding Biological Process: regulation of apoptosis; positive regulation of phosphoinositide 3-kinase cascade; positive regulation of protein kinase B signaling cascade; regulation of actin filament polymerization; positive regulation of peptidyl-tyrosine phosphorylation; positive regulation of granulocyte differentiation; positive regulation of transcription from RNA polymerase II promoter; positive regulation of peptidyl-serine phosphorylation Disease: Neutropenia, Severe Congenital, 3, Autosomal Recessive |
NCBI Summary: | 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] |
UniProt Code: | O00165 |
NCBI GenInfo Identifier: | 20141308 |
NCBI Gene ID: | 10456 |
NCBI Accession: | O00165.2 |
UniProt Secondary Accession: | O00165,Q5VYD5, Q5VYD7, Q96AU4, Q9BS80, A8W4W9, A8W4X0 B4DUJ7, |
UniProt Related Accession: | O00165 |
Molecular Weight: | 279 |
NCBI Full Name: | HCLS1-associated protein X-1 |
NCBI Synonym Full Names: | HCLS1 associated protein X-1 |
NCBI Official Symbol: | HAX1 |
NCBI Official Synonym Symbols: | SCN3; HS1BP1; HCLSBP1 |
NCBI Protein Information: | HCLS1-associated protein X-1; HAX-1; HSP1BP-1; HS1 binding protein; HS1-binding protein 1; HS1-associating protein X-1; HCLS1 (and PKD2) associated protein |
UniProt Protein Name: | HCLS1-associated protein X-1 |
UniProt Synonym Protein Names: | HS1-associating protein X-1; HAX-1; HS1-binding protein 1; HSP1BP-1 |
Protein Family: | HCLS1-associated protein |
UniProt Gene Name: | HAX1 |
UniProt Entry Name: | HAX1_HUMAN |