COG8 Antibody (PACO59309)
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주문- SKU:
- PACO59309
- Product type:
- Antibody
- Reactivity:
- Human
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Application:
- IHC
- Antibody type:
- Polyclonal
- Conjugation:
- Unconjugated
Frequently bought together:
Description
Antibody Name: | COG8 Antibody (PACO59309) |
Antibody SKU: | PACO59309 |
Size: | 50ug |
Host Species: | Rabbit |
Tested Applications: | ELISA, IHC |
Recommended Dilutions: | ELISA:1:2000-1:10000, IHC:1:20-1:200 |
Species Reactivity: | Human |
Immunogen: | Recombinant Human Conserved oligomeric Golgi complex subunit 8 protein (521-612AA) |
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 PACO59309 diluted at 1:100 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. |
Background: | Required for normal Golgi function. |
Synonyms: | Conserved oligomeric Golgi complex subunit 8 (COG complex subunit 8) (Component of oligomeric Golgi complex 8), COG8 |
UniProt Protein Function: | COG8: Required for normal Golgi function. Defects in COG8 are the cause of congenital disorder of glycosylation type 2H (CDG2H). CDGs are a family of severe inherited diseases caused by a defect in protein N- glycosylation. They are characterized by under-glycosylated serum proteins. These multisystem disorders present with a wide variety of clinical features, such as disorders of the nervous system development, psychomotor retardation, dysmorphic features, hypotonia, coagulation disorders, and immunodeficiency. The broad spectrum of features reflects the critical role of N-glycoproteins during embryonic development, differentiation, and maintenance of cell functions. Belongs to the COG8 family. |
UniProt Protein Details: | Protein type:Vesicle Chromosomal Location of Human Ortholog: 16q22.1 Cellular Component: Golgi membrane; membrane; Golgi transport complex Biological Process: protein transport Disease: Congenital Disorder Of Glycosylation, Type Iih |
NCBI Summary: | This gene encodes a protein that is a component of the conserved oligomeric Golgi (COG) complex, a multiprotein complex that plays a structural role in the Golgi apparatus, and is involved in intracellular membrane trafficking and glycoprotein modification. Mutations in this gene cause congenital disorder of glycosylation, type IIh, a disease that is characterized by under-glycosylated serum proteins, and whose symptoms include severe psychomotor retardation, failure to thrive, seizures, and dairy and wheat product intolerance. [provided by RefSeq, Jul 2008] |
UniProt Code: | Q96MW5 |
NCBI GenInfo Identifier: | 186928841 |
NCBI Gene ID: | 84342 |
NCBI Accession: | NP_115758.3 |
UniProt Secondary Accession: | Q96MW5,Q0VAK2, Q8WVV6, Q9H6F8, |
UniProt Related Accession: | Q96MW5 |
Molecular Weight: | 68,424 Da |
NCBI Full Name: | conserved oligomeric Golgi complex subunit 8 |
NCBI Synonym Full Names: | component of oligomeric golgi complex 8 |
NCBI Official Symbol: | COG8 |
NCBI Official Synonym Symbols: | DOR1; CDG2H |
NCBI Protein Information: | conserved oligomeric Golgi complex subunit 8; dependent on RIC1; COG complex subunit 8; conserved oligomeric golgi complex component 8 |
UniProt Protein Name: | Conserved oligomeric Golgi complex subunit 8 |
UniProt Synonym Protein Names: | Component of oligomeric Golgi complex 8 |
Protein Family: | Conserved oligomeric Golgi complex |
UniProt Gene Name: | COG8 |
UniProt Entry Name: | COG8_HUMAN |