algn-8

Caenorhabditis elegans

Predicted to enable dolichyl pyrophosphate Glc1Man9GlcNAc2 alpha-1,3-glucosyltransferase activity. Predicted to be involved in dolichol-linked oligosaccharide biosynthetic process. Predicted to be located in endoplasmic reticulum membrane. Human ortholog(s) of this gene implicated in congenital disorder of glycosylation Ih and polycystic liver disease. Is an ortholog of human ALG8 (ALG8 alpha-1,3-glucosyltransferase).

Ih

Drosophila melanogaster

I[[h]] channel (Ih) encodes a low-threshold, voltage-gated ion channel. It may influence excitatory postsynaptic potential kinetics and integration.

Alg8

Homo sapiens

This gene encodes a member of the ALG6/ALG8 glucosyltransferase family. The encoded protein catalyzes the addition of the second glucose residue to the lipid-linked oligosaccharide precursor for N-linked glycosylation of proteins. Mutations in this gene have been associated with congenital disorder of glycosylation type Ih (CDG-Ih). Alternatively spliced transcript variants encoding different isoforms have been identified. [provided by RefSeq, Jul 2008]

Idua

Rattus norvegicus

Enables L-iduronidase activity and signaling receptor binding activity. Involved in glycosaminoglycan catabolic process. Predicted to be located in extracellular space. Predicted to be active in lysosomal lumen. Biomarker of gastric ulcer. Human ortholog(s) of this gene implicated in Scheie syndrome; mucopolysaccharidosis I; mucopolysaccharidosis Ih; and mucopolysaccharidosis Ih/s. Orthologous to human IDUA (alpha-L-iduronidase); INTERACTS WITH (+)-schisandrin B; 2,3,7,8-Tetrachlorodibenzofuran; 3-chloropropane-1,2-diol.

Myo1h

Rattus norvegicus

Predicted to enable ATP binding activity; actin binding activity; and cytoskeletal motor activity. Predicted to be part of myosin complex. Human ortholog(s) of this gene implicated in congenital central hypoventilation syndrome. Orthologous to human MYO1H (myosin IH); INTERACTS WITH 2,2',4,4',5,5'-hexachlorobiphenyl (ortholog); 2,2',5,5'-tetrachlorobiphenyl (ortholog); 4,4'-sulfonyldiphenol (ortholog).

Alg2

Homo sapiens

This gene encodes a member of the glycosyltransferase 1 family. The encoded protein acts as an alpha 1,3 mannosyltransferase, mannosylating Man(2)GlcNAc(2)-dolichol diphosphate and Man(1)GlcNAc(2)-dolichol diphosphate to form Man(3)GlcNAc(2)-dolichol diphosphate. Defects in this gene have been associated with congenital disorder of glycosylation type Ih (CDG-Ii). Alternative splicing results in multiple transcript variants. [provided by RefSeq, Nov 2008]

Alg8

Rattus norvegicus

Predicted to enable dolichyl pyrophosphate Glc1Man9GlcNAc2 alpha-1,3-glucosyltransferase activity. Predicted to be involved in dolichol-linked oligosaccharide biosynthetic process and protein N-linked glycosylation via asparagine. Predicted to be active in endoplasmic reticulum membrane. Human ortholog(s) of this gene implicated in congenital disorder of glycosylation Ih and polycystic liver disease. Orthologous to human ALG8 (ALG8 alpha-1,3-glucosyltransferase); PARTICIPATES IN N-linked glycan biosynthetic pathway; INTERACTS WITH 2,3,7,8-tetrachlorodibenzodioxine; 2,6-dinitrotoluene; 6-propyl-2-thiouracil.