AMMECR1 Antibody (PACO57036)
- SKU:
- PACO57036
- Product Type:
- Antibody
- Reactivity:
- Human
- Host Species:
- Rabbit
- Isotype:
- IgG
- Applications:
- ELISA
- Applications:
- IHC
- Applications:
- IF
- Antibody Type:
- Polyclonal Antibody
- Conjugation:
- Unconjugated
Description
| Antibody Name: | AMMECR1 Antibody (PACO57036) |
| Antibody SKU: | PACO57036 |
| Size: | 50ug |
| Host Species: | Rabbit |
| Tested Applications: | ELISA, IHC, IF |
| Recommended Dilutions: | ELISA:1:2000-1:10000, IHC:1:200-1:500, IF:1:50-1:200 |
| Species Reactivity: | Human |
| Immunogen: | Recombinant Human AMME syndrome candidate gene 1 protein (146-248AA) |
| Form: | Liquid |
| Storage Buffer: | Preservative: 0.03% Proclin 300 Constituents: 50% Glycerol, 0.01M PBS, pH 7.4 |
| Purification Method: | >95%, Protein G purified |
| Clonality: | Polyclonal |
| Isotype: | IgG |
| Conjugate: | Non-conjugated |
 | IHC image of PACO57036 diluted at 1:200 and staining in paraffin-embedded human liver 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. |
 | Immunofluorescence staining of Hela cells with PACO57036 at 1:66, counter-stained with DAPI. The cells were fixed in 4% formaldehyde, permeabilized using 0.2% Triton X-100 and blocked in 10% normal Goat Serum. The cells were then incubated with the antibody overnight at 4°C. The secondary antibody was Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L). |
 | IHC image of PACO57036 diluted at 1:200 and staining in paraffin-embedded human testis tissue 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. |
| Background: | nucleus |
| Synonyms: | AMME syndrome candidate gene 1 protein, AMMECR1 |
| UniProt Protein Function: | AMMECR1: Defects in AMMECR1 are involved in Alport syndrome with mental retardation midface hypoplasia and elliptocytosis (ATS-MR). A X-linked contiguous gene deletion syndrome characterized by glomerulonephritis, deafness, mental retardation, midface hypoplasia and elliptocytosis. 4 isoforms of the human protein are produced by alternative splicing.Chromosomal Location of Human Ortholog: Xq22.3Molecular Function: protein bindingDisease: Amme Complex |
| UniProt Protein Details: | |
| NCBI Summary: | The exact function of this gene is not known, however, submicroscopic deletion of the X chromosome including this gene, COL4A5, and FACL4 genes, result in a contiguous gene deletion syndrome, the AMME complex (Alport syndrome, mental retardation, midface hypoplasia, and elliptocytosis). Alternatively spliced transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jan 2010] |
| UniProt Code: | Q9Y4X0 |
| NCBI GenInfo Identifier: | 48475039 |
| NCBI Gene ID: | 9949 |
| NCBI Accession: | Q9Y4X0.1 |
| UniProt Secondary Accession: | Q9Y4X0,Q5JYV9, Q6P9D8, Q8WX22, Q9UIQ8 |
| UniProt Related Accession: | Q9Y4X0 |
| Molecular Weight: | 24,581 Da |
| NCBI Full Name: | AMME syndrome candidate gene 1 protein |
| NCBI Synonym Full Names: | Alport syndrome, mental retardation, midface hypoplasia and elliptocytosis chromosomal region gene 1 |
| NCBI Official Symbol: | AMMECR1 |
| NCBI Official Synonym Symbols: | AMMERC1 |
| NCBI Protein Information: | AMME syndrome candidate gene 1 protein |
| UniProt Protein Name: | AMME syndrome candidate gene 1 protein |
| UniProt Synonym Protein Names: | |
| Protein Family: | AMMECR1-like protein |
| UniProt Gene Name: | AMMECR1 |
| UniProt Entry Name: | AMMR1_HUMAN |
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