COG8, Polyclonal Antibody

Goat Polyclonal to Human COG8; Human COG8; CDG2H; Dor1; COG complex subunit 8; Dependent on RIC1

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

Goat

Human COG8.

Immunoaffinity purified

Tris-buffered saline, pH 7.3, 0.5% BSA, 0.02% sodium azide

Store at -20 degree C. Minimize freezing and thawing.

Small volumes of anti-COG8 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

COG8 Antibody, CDG2H Antibody, Dor1 Antibody, COG complex subunit 8 Antibody, Dependent on RIC1 Antibody Description: COG8 is 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.

Immunohistochemistry (IHC) Paraffin, ELISA (EIA)

ELISA (1:16000)
IHC-P (5 ug/ml)

Anti-COG8 antibody IHC staining of human kidney. Immunohistochemistry of formalin-fixed, paraffin-embedded tissue after heat-induced antigen retrieval. Antibody concentration 5 ug/ml.

68,424 Da

component of oligomeric golgi complex 8

conserved oligomeric Golgi complex subunit 8

Conserved oligomeric Golgi complex subunit 8

COG complex subunit 8  [Similar Products]

COG8_HUMAN

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]

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.

Protein type: Vesicle

Chromosomal Location of Human Ortholog: 16q22.1

Cellular Component: Golgi membrane; Golgi transport complex; membrane

Molecular Function: protein binding

Biological Process: cellular protein metabolic process; ER to Golgi vesicle-mediated transport; intra-Golgi vesicle-mediated transport; post-translational protein modification; protein amino acid N-linked glycosylation via asparagine; protein transport

Disease: Congenital Disorder Of Glycosylation, Type Iih

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