COG8 Antibody (PACO08507)
- SKU:
- PACO08507
- Product type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Application:
- WB
- Antibody type:
- Polyclonal
- Conjugation:
- Unconjugated
Frequently bought together:
Description
抗体名: | COG8 Antibody (PACO08507) |
抗体コード: | PACO08507 |
サイズ: | 50ul |
宿主種: | Rabbit |
申し込み: | ELISA, WB |
推奨される希釈: | |
反応性: | Human, Mouse, Rat |
免疫原: | Human COG8 |
憲法: | Liquid |
ストレージバッファ: | PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles. |
精製方法: | Antigen Affinity Purified |
抗体のクローン性: | Polyclonal |
アイソタイプ: | IgG |
Conjugate: | Non-conjugated |
シノニム: | component of oligomeric golgi complex 8;COG8;DOR1;FLJ22315 ; |
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 |