Fibrillin microfibrils

Cay M. Kielty; Michael J. Sherratt; Andrew Marson; Clair Baldock

doi:10.1016/S0065-3233(05)70012-7

Fibrillin microfibrils

Cay M. Kielty
, Michael J. Sherratt
, Andrew Marson
, Clair Baldock

Research output: Contribution to journal › Article › peer-review

Abstract

Fibrillin microfibrils are widely distributed extracellular matrix assemblies that endow elastic and nonelastic connective tissues with long-range elasticity. They direct tropoelastin deposition during elastic fibrillogenesis and form an outer mantle for mature elastic fibers. Microfibril arrays are also abundant in dynamic tissues that do not express elastin, such as the ciliary zonules of the eye. Mutations in fibrillin-1 - the principal structural component of microfibrils - cause Marfan syndrome, a heritable disease with severe aortic, ocular, and skeletal defects. Isolated fibrillin-rich microfibrils have a complex 56 nm "beads-on-a-string" appearance; the molecular basis of their assembly and elastic properties, and their role in higher-order elastic fiber formation, remain incompletely understood. Copyright 2005, Elsevier Inc. All rights reserved.

Original language	English
Pages (from-to)	405-436
Number of pages	31
Journal	Advances in Protein Chemistry
Volume	70
DOIs	https://doi.org/10.1016/S0065-3233(05)70012-7
Publication status	Published - 2005

Keywords

physiology: Aging
Animals
Elasticity
genetics: Genetic Diseases, Inborn
Humans
chemistry: Microfibrils
biosynthesis: Microfilament Proteins

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10.1016/S0065-3233(05)70012-7

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title = "Fibrillin microfibrils",

abstract = "Fibrillin microfibrils are widely distributed extracellular matrix assemblies that endow elastic and nonelastic connective tissues with long-range elasticity. They direct tropoelastin deposition during elastic fibrillogenesis and form an outer mantle for mature elastic fibers. Microfibril arrays are also abundant in dynamic tissues that do not express elastin, such as the ciliary zonules of the eye. Mutations in fibrillin-1 - the principal structural component of microfibrils - cause Marfan syndrome, a heritable disease with severe aortic, ocular, and skeletal defects. Isolated fibrillin-rich microfibrils have a complex 56 nm {"}beads-on-a-string{"} appearance; the molecular basis of their assembly and elastic properties, and their role in higher-order elastic fiber formation, remain incompletely understood. Copyright 2005, Elsevier Inc. All rights reserved.",

keywords = "physiology: Aging, Animals, Elasticity, genetics: Genetic Diseases, Inborn, Humans, chemistry: Microfibrils, biosynthesis: Microfilament Proteins",

author = "Kielty, \{Cay M.\} and Sherratt, \{Michael J.\} and Andrew Marson and Clair Baldock",

year = "2005",

doi = "10.1016/S0065-3233(05)70012-7",

language = "English",

volume = "70",

pages = "405--436",

journal = "Advances in Protein Chemistry",

issn = "0065-3233",

publisher = "Elsevier BV",

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TY - JOUR

T1 - Fibrillin microfibrils

AU - Kielty, Cay M.

AU - Sherratt, Michael J.

AU - Marson, Andrew

AU - Baldock, Clair

PY - 2005

Y1 - 2005

N2 - Fibrillin microfibrils are widely distributed extracellular matrix assemblies that endow elastic and nonelastic connective tissues with long-range elasticity. They direct tropoelastin deposition during elastic fibrillogenesis and form an outer mantle for mature elastic fibers. Microfibril arrays are also abundant in dynamic tissues that do not express elastin, such as the ciliary zonules of the eye. Mutations in fibrillin-1 - the principal structural component of microfibrils - cause Marfan syndrome, a heritable disease with severe aortic, ocular, and skeletal defects. Isolated fibrillin-rich microfibrils have a complex 56 nm "beads-on-a-string" appearance; the molecular basis of their assembly and elastic properties, and their role in higher-order elastic fiber formation, remain incompletely understood. Copyright 2005, Elsevier Inc. All rights reserved.

AB - Fibrillin microfibrils are widely distributed extracellular matrix assemblies that endow elastic and nonelastic connective tissues with long-range elasticity. They direct tropoelastin deposition during elastic fibrillogenesis and form an outer mantle for mature elastic fibers. Microfibril arrays are also abundant in dynamic tissues that do not express elastin, such as the ciliary zonules of the eye. Mutations in fibrillin-1 - the principal structural component of microfibrils - cause Marfan syndrome, a heritable disease with severe aortic, ocular, and skeletal defects. Isolated fibrillin-rich microfibrils have a complex 56 nm "beads-on-a-string" appearance; the molecular basis of their assembly and elastic properties, and their role in higher-order elastic fiber formation, remain incompletely understood. Copyright 2005, Elsevier Inc. All rights reserved.

KW - physiology: Aging

KW - Animals

KW - Elasticity

KW - genetics: Genetic Diseases, Inborn

KW - Humans

KW - chemistry: Microfibrils

KW - biosynthesis: Microfilament Proteins

U2 - 10.1016/S0065-3233(05)70012-7

DO - 10.1016/S0065-3233(05)70012-7

M3 - Article

C2 - 15837522

SN - 0065-3233

VL - 70

SP - 405

EP - 436

JO - Advances in Protein Chemistry

JF - Advances in Protein Chemistry

ER -

Fibrillin microfibrils

Abstract

Keywords

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