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Article

Biology of anchoring fibrils: lessons from dystrophic epidermolysis bullosa.

Matrix biology : journal of the International Society for Matrix Biology - 1 Feb 1999

Bruckner-Tuderman L, Höpfner B, Hammami-Hauasli N

Abstract excerpt

Anchoring fibrils are adhesive suprastructures that ensure the connection of the epidermal basement membrane with the dermal extracellular matrix. The fibrils represent polymers of collagen VII, the major structural fibril component, but may also contain other proteins. Remarkable progress has been made in the last few years in understanding the functions of skin basement membrane components including the...

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Methodology Data sources Editorial policy Privacy Delete account & data About Contact Helena Bioinformatics

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Source

Identifiers

PMID: 10367730
DOI: 10.1016/s0945-053x(98)00007-9

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

Related research

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Biology of anchoring fibrils: lessons from dystrophic epidermolysis bullosa.PMID 10367730Read ↗

Type VII collagen: the anchoring fibril protein at fault in dystrophic epidermolysis bullosa.Semantic proximity Read ↗

Characterization of 18 new mutations in COL7A1 in recessive dystrophic epidermolysis bullosa provides evidence for distinct molecular mechanisms underlying defective anchoring fibril formation.Semantic proximity Read ↗

Molecular basis of dystrophic epidermolysis bullosa: mutations in the type VII collagen gene (COL7A1).Semantic proximity Read ↗

Targeted inactivation of the type VII collagen gene (Col7a1) in mice results in severe blistering phenotype: a model for recessive dystrophic epidermolysis bullosa.Semantic proximity Read ↗

Type VII Collagen Disorders Simplified.Semantic proximity Read ↗

Observations of dystrophic epidermolysis bullosa patients with collagen VII NC2 truncation provide new insights into anchoring fibril assembly.Semantic proximity Read ↗

Some, but not all, glycine substitution mutations in COL7A1 result in intracellular accumulation of collagen VII, loss of anchoring fibrils, and skin blistering.Semantic proximity Read ↗

Molecular basis for the dystrophic forms of epidermolysis bullosa: mutations in the type VII collagen gene.Semantic proximity Read ↗

Clinicopathological correlations of compound heterozygous COL7A1 mutations in recessive dystrophic epidermolysis bullosa.Semantic proximity Read ↗

Premature termination codons in the type VII collagen gene (COL7A1) underlie severe, mutilating recessive dystrophic epidermolysis bullosa.Semantic proximity Read ↗

A missense mutation in type VII collagen in two affected siblings with recessive dystrophic epidermolysis bullosa.Semantic proximity Read ↗

Molecular basis of recessive dystrophic epidermolysis bullosa: genotype/phenotype correlation in a case of moderate clinical severity.Semantic proximity Read ↗

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