A novel dystrophin/utrophin-associated protein is an enzymatically inactive member of the phosphoglucomutase superfamily

Eur J Biochem. 1996 Jan 15;235(1-2):103-13. doi: 10.1111/j.1432-1033.1996.00103.x.


A 60-kDa protein localised in adherens-type cellular junctions, and previously called aciculin, has been found to interact with the cytoskeletal proteins dystrophin and utrophin [Belkin, A. M. & Burridge, K. (1995) J. Biol. Chem. 270, 6328-6337]. In this study, we report the complete sequence of this protein, and show that it is a novel member of the phosphoglucomutase (PGM) family of proteins. The PGM-related protein (PGM-RP), which contains 506 amino acids (55.6 kDa), is smaller than PGM1 (566 amino acids, 61 kDa). The active site consensus sequences of prokaryotic and eukaryotic mutases are not conserved in PGM-RP, a finding consistent with the lack of enzymatic activity of PGM-RP in vitro, and the absence of a phosphorylated intermediate in vivo. The organisation of the PGM-RP gene is essentially identical to that of PGM1. We propose that the PGM-RP gene, which we have mapped to human chromosome 9qcen-q13, evolved from the PGM1 gene, and encodes a protein with a structural rather than an enzymatic role. PGM-RP is expressed predominantly in muscle with the highest levels in smooth muscle. The significance of the interaction between dystrophin/utrophin and an increasing number of cytoplasmic proteins including PGM-RP remains to be explored.

Publication types

  • Comparative Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Amino Acid Sequence
  • Animals
  • Base Sequence
  • Binding Sites / genetics
  • Biological Evolution
  • Chromosome Mapping
  • Chromosomes, Human, Pair 9 / genetics
  • Consensus Sequence
  • Cytoskeletal Proteins / chemistry
  • Cytoskeletal Proteins / genetics
  • Cytoskeletal Proteins / metabolism*
  • DNA Primers / genetics
  • DNA, Complementary / genetics
  • Dystrophin / metabolism*
  • Exons
  • Female
  • Humans
  • In Vitro Techniques
  • Membrane Proteins*
  • Molecular Sequence Data
  • Molecular Weight
  • Muscle Proteins / chemistry
  • Muscle Proteins / genetics
  • Muscle Proteins / metabolism
  • Muscle, Smooth / metabolism
  • Phosphoglucomutase / chemistry
  • Phosphoglucomutase / genetics
  • Phosphoglucomutase / metabolism*
  • Sequence Homology, Amino Acid
  • Tissue Distribution
  • Utrophin


  • Cytoskeletal Proteins
  • DNA Primers
  • DNA, Complementary
  • Dystrophin
  • Membrane Proteins
  • Muscle Proteins
  • PGM5 protein, human
  • Utrn protein, mouse
  • Utrophin
  • Phosphoglucomutase

Associated data

  • GENBANK/L40933
  • GENBANK/L42902
  • GENBANK/L42903
  • GENBANK/L42904
  • GENBANK/L42905
  • GENBANK/L42907