Substitution of cysteine for glycine-alpha 1-691 in the pro alpha 1(I) chain of type I procollagen in a proband with lethal osteogenesis imperfecta destabilizes the triple helix at a site C-terminal to the substitution.

doi:10.1042/bj2790747

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Article

Substitution of cysteine for glycine-alpha 1-691 in the pro alpha 1(I) chain of type I procollagen in a proband with lethal osteogenesis imperfecta destabilizes the triple helix at a site C-terminal to the substitution.

The Biochemical journal - 1 Nov 1991

Steinmann B, Westerhausen A, Constantinou C D, Superti-Furga A, Prockop D J

Abstract excerpt

Skin fibroblasts from a proband with lethal osteogenesis imperfecta synthesized a type I procollagen containing a cysteine residue in the alpha 1(I) helical domain. Assay of thermal stability of the triple helix by proteinase digestion demonstrated a decreased temperature for thermal unfolding of...

Topics

Alleles
Base Sequence
Cells, Cultured
Cysteine
Female
Fibroblasts
Glycine
Humans
Infant, Newborn
Molecular Sequence Data
Mutation
Nucleic Acid Hybridization
Osteogenesis Imperfecta
Prenatal Diagnosis

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Procollagen

Protein Conformation

Skin

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Identifiers

PMID: 1953667
DOI: 10.1042/bj2790747
PMCID: PMC1151509

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 30 Aug 2026. Prepared record publication-1953667-2026-08-30.1. Manifest publication-graph-cohort-2026-08-30.1.

Source receipt: PubMed via Helena Literature Corpus, retrieved 30 Aug 2026.