Inactivation of tensin3 in mice results in growth retardation and postnatal lethality

Ming Ko Chiang, Yi Chun Liao, Yasuko Kuwabara, Su Hao Lo

Research output: Contribution to journalArticle

23 Citations (Scopus)

Abstract

Tensin family is a group of focal adhesion proteins that interact with integrins, actin, and phosphotyrosine-containing proteins. To explore the in vivo functions of a new member of the family, tensin3, we have generated mutant mice with a disrupted tensin3 gene. Inactivation of tensin3 resulted in growth retardation and postnatal lethality in one third of the homozygous mutants. Histological analysis of those mutants showed incomplete development of the small intestine, lung, and bone. Villus formation in the small intestine was affected and cells migrated slower in the runt mutants. Their lungs also displayed enlarged air space suggesting defects in alveogenesis. In addition, the resting zone was thicker and fewer proliferating cells were present in the growth plates of tensin3-/- tibiae. These observations indicate that tensin3 is essential for normal development and functions of the small intestine, lung, and bone. These phenotypes of the runt tensin3-/- mice are similar to some clinical features of Silver-Russell syndrome (SRS) which is a genetically inherited defect. About 10% of SRS cases have been linked to abnormality in chromosome 7p11.2-13, where human tensin3 gene is located, suggesting a potential link between tensin3 and SRS.

Original languageEnglish (US)
Pages (from-to)368-377
Number of pages10
JournalDevelopmental Biology
Volume279
Issue number2
DOIs
StatePublished - Mar 15 2005

Fingerprint

Silver-Russell Syndrome
Small Intestine
Lung
Growth
Bone and Bones
Chromosomes, Human, Pair 13
Phosphotyrosine
Focal Adhesions
Growth Plate
Tibia
Integrins
Genes
Actins
Proteins
Air
Phenotype

Keywords

  • Alveogenesis
  • Focal adhesion
  • Growth plates
  • Growth retardation
  • Postnatal lethality
  • Silver-Russell syndrome
  • Tensin

ASJC Scopus subject areas

  • Developmental Biology

Cite this

Inactivation of tensin3 in mice results in growth retardation and postnatal lethality. / Chiang, Ming Ko; Liao, Yi Chun; Kuwabara, Yasuko; Lo, Su Hao.

In: Developmental Biology, Vol. 279, No. 2, 15.03.2005, p. 368-377.

Research output: Contribution to journalArticle

Chiang, Ming Ko ; Liao, Yi Chun ; Kuwabara, Yasuko ; Lo, Su Hao. / Inactivation of tensin3 in mice results in growth retardation and postnatal lethality. In: Developmental Biology. 2005 ; Vol. 279, No. 2. pp. 368-377.
@article{7798e27b811f402baa3075812e7ad0d4,
title = "Inactivation of tensin3 in mice results in growth retardation and postnatal lethality",
abstract = "Tensin family is a group of focal adhesion proteins that interact with integrins, actin, and phosphotyrosine-containing proteins. To explore the in vivo functions of a new member of the family, tensin3, we have generated mutant mice with a disrupted tensin3 gene. Inactivation of tensin3 resulted in growth retardation and postnatal lethality in one third of the homozygous mutants. Histological analysis of those mutants showed incomplete development of the small intestine, lung, and bone. Villus formation in the small intestine was affected and cells migrated slower in the runt mutants. Their lungs also displayed enlarged air space suggesting defects in alveogenesis. In addition, the resting zone was thicker and fewer proliferating cells were present in the growth plates of tensin3-/- tibiae. These observations indicate that tensin3 is essential for normal development and functions of the small intestine, lung, and bone. These phenotypes of the runt tensin3-/- mice are similar to some clinical features of Silver-Russell syndrome (SRS) which is a genetically inherited defect. About 10{\%} of SRS cases have been linked to abnormality in chromosome 7p11.2-13, where human tensin3 gene is located, suggesting a potential link between tensin3 and SRS.",
keywords = "Alveogenesis, Focal adhesion, Growth plates, Growth retardation, Postnatal lethality, Silver-Russell syndrome, Tensin",
author = "Chiang, {Ming Ko} and Liao, {Yi Chun} and Yasuko Kuwabara and Lo, {Su Hao}",
year = "2005",
month = "3",
day = "15",
doi = "10.1016/j.ydbio.2004.12.027",
language = "English (US)",
volume = "279",
pages = "368--377",
journal = "Developmental Biology",
issn = "0012-1606",
publisher = "Academic Press Inc.",
number = "2",

}

TY - JOUR

T1 - Inactivation of tensin3 in mice results in growth retardation and postnatal lethality

AU - Chiang, Ming Ko

AU - Liao, Yi Chun

AU - Kuwabara, Yasuko

AU - Lo, Su Hao

PY - 2005/3/15

Y1 - 2005/3/15

N2 - Tensin family is a group of focal adhesion proteins that interact with integrins, actin, and phosphotyrosine-containing proteins. To explore the in vivo functions of a new member of the family, tensin3, we have generated mutant mice with a disrupted tensin3 gene. Inactivation of tensin3 resulted in growth retardation and postnatal lethality in one third of the homozygous mutants. Histological analysis of those mutants showed incomplete development of the small intestine, lung, and bone. Villus formation in the small intestine was affected and cells migrated slower in the runt mutants. Their lungs also displayed enlarged air space suggesting defects in alveogenesis. In addition, the resting zone was thicker and fewer proliferating cells were present in the growth plates of tensin3-/- tibiae. These observations indicate that tensin3 is essential for normal development and functions of the small intestine, lung, and bone. These phenotypes of the runt tensin3-/- mice are similar to some clinical features of Silver-Russell syndrome (SRS) which is a genetically inherited defect. About 10% of SRS cases have been linked to abnormality in chromosome 7p11.2-13, where human tensin3 gene is located, suggesting a potential link between tensin3 and SRS.

AB - Tensin family is a group of focal adhesion proteins that interact with integrins, actin, and phosphotyrosine-containing proteins. To explore the in vivo functions of a new member of the family, tensin3, we have generated mutant mice with a disrupted tensin3 gene. Inactivation of tensin3 resulted in growth retardation and postnatal lethality in one third of the homozygous mutants. Histological analysis of those mutants showed incomplete development of the small intestine, lung, and bone. Villus formation in the small intestine was affected and cells migrated slower in the runt mutants. Their lungs also displayed enlarged air space suggesting defects in alveogenesis. In addition, the resting zone was thicker and fewer proliferating cells were present in the growth plates of tensin3-/- tibiae. These observations indicate that tensin3 is essential for normal development and functions of the small intestine, lung, and bone. These phenotypes of the runt tensin3-/- mice are similar to some clinical features of Silver-Russell syndrome (SRS) which is a genetically inherited defect. About 10% of SRS cases have been linked to abnormality in chromosome 7p11.2-13, where human tensin3 gene is located, suggesting a potential link between tensin3 and SRS.

KW - Alveogenesis

KW - Focal adhesion

KW - Growth plates

KW - Growth retardation

KW - Postnatal lethality

KW - Silver-Russell syndrome

KW - Tensin

UR - http://www.scopus.com/inward/record.url?scp=14544299287&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=14544299287&partnerID=8YFLogxK

U2 - 10.1016/j.ydbio.2004.12.027

DO - 10.1016/j.ydbio.2004.12.027

M3 - Article

C2 - 15733665

AN - SCOPUS:14544299287

VL - 279

SP - 368

EP - 377

JO - Developmental Biology

JF - Developmental Biology

SN - 0012-1606

IS - 2

ER -