CEP135 Antibody (PACO57192)
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주문- SKU:
- PACO57192
- Product type:
- Antibody
- Reactivity:
- Human
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Application:
- IF
- Antibody type:
- Polyclonal
- Conjugation:
- Unconjugated
Frequently bought together:
Description
Antibody Name: | CEP135 Antibody (PACO57192) |
Antibody SKU: | PACO57192 |
Size: | 50ug |
Host Species: | Rabbit |
Tested Applications: | ELISA, IF |
Recommended Dilutions: | ELISA:1:2000-1:10000, IF:1:50-1:200 |
Species Reactivity: | Human |
Immunogen: | Recombinant Human Centrosomal protein of 135 kDa protein (94-195AA) |
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 |
Immunofluorescence staining of A549 cells with PACO57192 at 1:100, 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). |
Background: | Centrosomal protein involved in centriole biogenesis. Acts as a scaffolding protein during early centriole biogenesis. Required for the targeting of centriole satellite proteins to centrosomes such as of PCM1, SSX2IP and CEP290 and recruitment of WRAP73 to centrioles. Also required for centriole-centriole cohesion during interphase by acting as a platform protein for CEP250 at the centriole. |
Synonyms: | Centrosomal protein of 135 kDa (Cep135) (Centrosomal protein 4), CEP135, CEP4 KIAA0635 |
UniProt Protein Function: | CEP135: Centrosomal protein involved in centriole biogenesis. Acts as a scaffolding protein during early centriole biogenesis. Also required for centriole-centriole cohesion during interphase by acting as a platform protein for CEP250 at the centriole. Defects in CEP135 are the cause of microcephaly, primary, type 8 (MCPH8). MCPH8 is a disease defined as a head circumference more than 3 standard deviations below the age- related mean. Brain weight is markedly reduced and the cerebral cortex is disproportionately small. Despite this marked reduction in size, the gyral pattern is relatively well preserved, with no major abnormality in cortical architecture. Affected individuals are mentally retarded. Primary microcephaly is further defined by the absence of other syndromic features or significant neurological deficits due to degenerative brain disorder. Belongs to the CEP135/TSGA10 family. 2 isoforms of the human protein are produced by alternative splicing. |
UniProt Protein Details: | Protein type:Unknown function Chromosomal Location of Human Ortholog: 4q12 Cellular Component: centriole; centrosome; cytosol Molecular Function:protein binding; protein C-terminus binding Biological Process: centriole replication; centriole-centriole cohesion; G2/M transition of mitotic cell cycle; mitotic cell cycle; organelle organization and biogenesis Disease: Microcephaly 8, Primary, Autosomal Recessive |
NCBI Summary: | This gene encodes a centrosomal protein, which acts as a scaffolding protein during early centriole biogenesis, and is also required for centriole-centriole cohesion during interphase. Mutations in this gene are associated with autosomal recessive primary microcephaly-8. [provided by RefSeq, Jun 2012] |
UniProt Code: | Q66GS9 |
NCBI GenInfo Identifier: | 296434460 |
NCBI Gene ID: | 9662 |
NCBI Accession: | Q66GS9.2 |
UniProt Secondary Accession: | Q66GS9,O75130, Q58F25, Q9H8H7, B2RMY0, |
UniProt Related Accession: | Q66GS9 |
Molecular Weight: | 28,910 Da |
NCBI Full Name: | Centrosomal protein of 135 kDa |
NCBI Synonym Full Names: | centrosomal protein 135 |
NCBI Official Symbol: | CEP135 |
NCBI Official Synonym Symbols: | CEP4; MCPH8; KIAA0635 |
NCBI Protein Information: | centrosomal protein of 135 kDa |
UniProt Protein Name: | Centrosomal protein of 135 kDa |
UniProt Synonym Protein Names: | Centrosomal protein 4 |
Protein Family: | Centrosomal protein |
UniProt Gene Name: | CEP135 |
UniProt Entry Name: | CP135_HUMAN |