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. 2000 Feb 18;468(1):79-83.
doi: 10.1016/s0014-5793(00)01195-9.

Biosynthesis of dystroglycan: processing of a precursor propeptide

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Free article

Biosynthesis of dystroglycan: processing of a precursor propeptide

K H Holt et al. FEBS Lett. .
Free article

Abstract

Dystroglycan is a cytoskeleton-linked extracellular matrix receptor expressed in many cell types. Dystroglycan is composed of alpha- and beta-subunits which are encoded by a single mRNA. Using a heterologous mammalian expression system, we provide the first biochemical evidence of the alpha/beta-dystroglycan precursor propeptide prior to enzymatic cleavage. This 160 kDa dystroglycan propeptide is processed into alpha- and beta-dystroglycan (120 kDa and 43 kDa, respectively). We also demonstrate that the precursor propeptide is glycosylated and that blockade of asparagine-linked (N-linked) glycosylation did not prevent the cleavage of the dystroglycan precursor peptide. However, inhibition of N-linked glycosylation results in aberrant trafficking of the alpha- and beta-dystroglycan subunits to the plasma membrane. Thus, dystroglycan is synthesized as a precursor propeptide that is post-translationally cleaved and differentially glycosylated to yield alpha- and beta-dystroglycan.

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