Gmppb - GDP-mannose pyrophosphorylase B

Predicted to enable mannose-1-phosphate guanylyltransferase activity. Predicted to be involved in GDP-mannose biosynthetic process; cell wall mannoprotein biosynthetic process; and protein glycosylation. Located in mitochondrion. Is expressed in several structures, including alimentary system; genitourinary system; nervous system; respiratory system; and sensory organ. Human ortholog(s) of this gene implicated in autosomal recessive limb-girdle muscular dystrophy type 2T; congenital muscular dystrophy-dystroglycanopathy A14; and muscular dystrophy-dystroglycanopathy. Orthologous to human GMPPB (GDP-mannose pyrophosphorylase B). [provided by Alliance of Genome Resources, Apr 2022]