The Tight skin mouse: demonstration of mutant fibrillin-1 production and assembly into abnormal microfibrils. (Q36255202)

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9 November 2019

title

The Tight skin mouse: demonstration of mutant fibrillin-1 production and assembly into abnormal microfibrils (English)

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9 November 2019

2

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9 November 2019

Michael J Sherratt

4

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9 November 2019

author name string

C M Kielty

1

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9 November 2019

L D Siracusa

3

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9 November 2019

R Peters

5

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9 November 2019

C A Shuttleworth

6

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9 November 2019

S A Jimenez

7

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9 November 2019

language of work or name

English

0 references

publication date

1 March 1998

1 reference

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9 November 2019

number of pages

8

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inferred from page(s)

published in

Journal of Cell Biology

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9 November 2019

volume

140

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9 November 2019

issue

5

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9 November 2019

page(s)

1159-1166

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Fibrillln mutations in Marfan syndrome and related phenotypes

9 November 2019

cites work

1 reference

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Latent transforming growth factor-beta 1 and its binding protein are components of extracellular matrix microfibrils

30 September 2017

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Solution structure of a pair of calcium-binding epidermal growth factor-like domains: implications for the Marfan syndrome and other genetic disorders

30 September 2017

1 reference

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Fibrillin-1: organization in microfibrils and structural properties

30 September 2017

1 reference

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Further characterization of proteins associated with elastic fiber microfibrils including the molecular cloning of MAGP-2 (MP25)

30 September 2017

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Bovine latent transforming growth factor beta 1-binding protein 2: molecular cloning, identification of tissue isoforms, and immunolocalization to elastin-associated microfibrils

30 September 2017

1 reference

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Genomic organization of the sequence coding for fibrillin, the defective gene product in Marfan syndrome.

30 September 2017

1 reference

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Fibrillin Binds Calcium and Is Coded by cDNAs That Reveal a Multidomain Structure and Alternatively Spliced Exons at the 5′ End

30 September 2017

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Fibrillin-containing microfibrils: Structure and function in health and disease

30 September 2017

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Elastin-Associated Microfibrils and Microfibrillar Proteins

30 September 2017

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Fibrillin, a new 350-kD glycoprotein, is a component of extracellular microfibrils

30 September 2017

1 reference

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Connective tissue microfibrils. Isolation and characterization of three large pepsin-resistant domains of fibrillin.

30 September 2017

1 reference

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Purification and partial characterization of fibrillin, a cysteine-rich structural component of connective tissue microfibrils

30 September 2017

1 reference

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Tight-skin, a new mutation of the mouse causing excessive growth of connective tissue and skeleton

30 September 2017

1 reference

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Proteoglycans associated with the ciliary zonule of the rat eye: a histochemical and immunocytochemical study

30 September 2017

1 reference

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Abnormal fibrillin assembly by dermal fibroblasts from two patients with Marfan syndrome

29 June 2018

1 reference

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Restrictive dermopathy: a newly recognized autosomal recessive skin dysplasia

29 June 2018

1 reference

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Tsk mice with genetic emphysema. Right ventricular hypertrophy occurs without hypertrophy of muscular pulmonary arteries or muscularization of arterioles.

29 June 2018

1 reference

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Fibrillin: evidence that chondroitin sulphate proteoglycans are components of microfibrils and associate with newly synthesised monomers

29 June 2018

1 reference

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Truncated profibrillin of a Marfan patient is of apparent similar size as fibrillin: intracellular retention leads to over-N-glycosylation

21 January 2018

1 reference

https://api.crossref.org/works/10.1083/JCB.140.5.1159

Calcium stabilizes fibrillin-1 against proteolytic degradation.

21 January 2018

1 reference

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Expression of fibrillar types I and III and basement membrane collagen type IV genes in myocardium of tight skin mouse

21 January 2018

1 reference

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Case of the month: a newborn with tight skin and joint contractures

21 January 2018

1 reference

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A biochemical and morphological investigation of the early development of genetic emphysema in tight-skin mice

21 January 2018

1 reference

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Morphology of sheet-like assemblies of pN-collagen, pC-collagen and procollagen studied by scanning transmission electron microscopy mass measurements

21 January 2018

1 reference

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Scleroderma-like alterations in collagen metabolism occurring in the TSK (tight skin) mouse

21 January 2018

1 reference

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The role of calcium in the organization of fibrillin microfibrils

21 January 2018

1 reference

https://api.crossref.org/works/10.1083/JCB.140.5.1159

A 16-month study of the development of genetic emphysema in tight-skin mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1083/JCB.140.5.1159

Collagenous abnormalities in the heart of the tight-skin mouse

21 January 2018

1 reference

https://api.crossref.org/works/10.1083/JCB.140.5.1159

Hereditary emphysema in the tight-skin mouse. Evaluation of pathogenesis

21 January 2018

1 reference

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Scanning transmission electron microscopy mass analysis of fibrillin-containing microfibrils from foetal elastic tissues

21 January 2018

1 reference

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A tandem duplication within the fibrillin 1 gene is associated with the mouse tight skin mutation

21 January 2018

1 reference

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Glycosaminoglycan content in skin of the tight-skin mouse

21 January 2018

1 reference

PubMed

https://pubmed.ncbi.nlm.nih.gov/9490728

12 December 2020

inferred from PubMed ID database lookup

Increased collagen biosynthesis and increased expression of type I and type III procollagen genes in tight skin (TSK) mouse fibroblasts

1 reference

PubMed

https://pubmed.ncbi.nlm.nih.gov/9490728

12 December 2020

inferred from PubMed ID database lookup

Ehlers-Danlos syndrome type VIIB. Morphology of type I collagen fibrils formed in vivo and in vitro is determined by the conformation of the retained N-propeptide

1 reference

PubMed

https://pubmed.ncbi.nlm.nih.gov/9490728

12 December 2020

inferred from PubMed ID database lookup

Identifiers

DOI

10.1083/JCB.140.5.1159

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:9490728 AND SRC:MED&resulttype=core&format=json

9 November 2019

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:9490728 AND SRC:MED&resulttype=core&format=json

9 November 2019

PubMed publication ID

9490728

1 reference