Fine structure of extracellular matrix and basal laminae in two types of abnormal collagen production

L-proline analog-treated otocyst cultures and disproportionate micromelia (Dmm/Dmm) mutants

Diana Berggren, Dorothy Frenz, Vera Galinovic-Schwartz, Thomas R Van De Water

Research output: Contribution to journalArticle

13 Citations (Scopus)

Abstract

L-Azetidine-2-carboxylic acid (LACA), a naturally occurring vegetable imino acid, can be incorporated into mammalian proteins in place of proline, thereby eliciting an inhibitory effect on collagen secretion. Exposure of explants of the embryonic mouse inner ear to LACA reduces the number of collagen fibrils in the otic capsule, gives rise to a dose-dependent derangement of the basal lamina, and ultimately results in dysmorphogenesis and retarded differentiation of the inner ear. Disproportionate micromelia (Dmm) is an incomplete dominant form of dwarfism characterized by a reduced quantity of type II collagen in the cartilaginous extracellular matrix (ECM). Abnormal morphogenesis in homozygotic Dmm mice resembles the abnormal morphogenesis observed in LACA-exposed otic explants, resulting in malformed inner ears with a bulky cartilaginous capsule and a lack or reduction of defined perilymphatic spaces (Van De Water and Galinovic-Schwartz, 1987). In this study, we examined by ultrastructural analysis LACA-exposed otic explants and inner ears of Dmm/Dmm mouse embryos for abnormalities in the collagenous constituents of the basal laminae and capsular ECM. We demonstrate, in comparison to normal embryonic mouse inner ears, a reduction in collagen fibrils and irregular cytodifferentiation of chondrocytes in the ECM of LACA-exposed and Dmm/Dmm inner ears as well as in the basal laminae of LACA-exposed specimens. In addition, we provide evidence of dysmorphogenesis of the otic capsule and perilymphatic spaces in LACA-exposed explants. Moreover, while previous studies demonstrated the anomalous development of sensory structures in otocyst explants following LACA exposure, in this study we provide evidence of the normal morphogenesis of otic epithelial-derived sensory structures in homozygotic Dmm/Dmm mouse embryos.

Original languageEnglish
Pages (from-to)125-135
Number of pages11
JournalHearing Research
Volume107
Issue number1-2
DOIs
StatePublished - May 1 1997
Externally publishedYes

Fingerprint

Azetidinecarboxylic Acid
Basement Membrane
Proline
Extracellular Matrix
Inner Ear
Collagen
Ear
Morphogenesis
Capsules
Embryonic Structures
Imino Acids
Dwarfism
Collagen Type II
Chondrocytes
Vegetables

Keywords

  • Basal lamina
  • Collagen
  • Disproportionate micromelia
  • Extracellular matrix
  • L-Azetidine-2-carboxylic acid
  • Mouse
  • Otic anlage

ASJC Scopus subject areas

  • Sensory Systems

Cite this

Fine structure of extracellular matrix and basal laminae in two types of abnormal collagen production : L-proline analog-treated otocyst cultures and disproportionate micromelia (Dmm/Dmm) mutants. / Berggren, Diana; Frenz, Dorothy; Galinovic-Schwartz, Vera; Van De Water, Thomas R.

In: Hearing Research, Vol. 107, No. 1-2, 01.05.1997, p. 125-135.

Research output: Contribution to journalArticle

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abstract = "L-Azetidine-2-carboxylic acid (LACA), a naturally occurring vegetable imino acid, can be incorporated into mammalian proteins in place of proline, thereby eliciting an inhibitory effect on collagen secretion. Exposure of explants of the embryonic mouse inner ear to LACA reduces the number of collagen fibrils in the otic capsule, gives rise to a dose-dependent derangement of the basal lamina, and ultimately results in dysmorphogenesis and retarded differentiation of the inner ear. Disproportionate micromelia (Dmm) is an incomplete dominant form of dwarfism characterized by a reduced quantity of type II collagen in the cartilaginous extracellular matrix (ECM). Abnormal morphogenesis in homozygotic Dmm mice resembles the abnormal morphogenesis observed in LACA-exposed otic explants, resulting in malformed inner ears with a bulky cartilaginous capsule and a lack or reduction of defined perilymphatic spaces (Van De Water and Galinovic-Schwartz, 1987). In this study, we examined by ultrastructural analysis LACA-exposed otic explants and inner ears of Dmm/Dmm mouse embryos for abnormalities in the collagenous constituents of the basal laminae and capsular ECM. We demonstrate, in comparison to normal embryonic mouse inner ears, a reduction in collagen fibrils and irregular cytodifferentiation of chondrocytes in the ECM of LACA-exposed and Dmm/Dmm inner ears as well as in the basal laminae of LACA-exposed specimens. In addition, we provide evidence of dysmorphogenesis of the otic capsule and perilymphatic spaces in LACA-exposed explants. Moreover, while previous studies demonstrated the anomalous development of sensory structures in otocyst explants following LACA exposure, in this study we provide evidence of the normal morphogenesis of otic epithelial-derived sensory structures in homozygotic Dmm/Dmm mouse embryos.",
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