Accelerated mortality from hydrocephalus and pneumonia in mice with a combined deficiency of SPAG6 and SPAG16L reveals a functional interrelationship between the two central apparatus proteins.

Accelerated mortality from hydrocephalus and pneumonia in mice with a combined deficiency of SPAG6 and SPAG16L reveals a functional interrelationship between the two central apparatus proteins. Research Abstract Details 

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Accelerated mortality from hydrocephalus and pneumonia in mice with a combined deficiency of SPAG6 and SPAG16L reveals a functional interrelationship between the two central apparatus proteins. Abstract Text:

Zhibing Zhang,Waixing Tang,Rong Zhou,Xuening Shen,Zhangyong Wei,Aatish M Patel,John T Povlishock,Jean Bennett,Jerome F Strauss,

SPAG6 and SPAG16L are proteins localized to the "9+2" axoneme central apparatus. Both are essential for sperm motility and male fertility. These two proteins are also expressed in other tissues containing ciliated cells, such as brain and lung. To study the effects of combined deficiency of these two proteins, a double mutant mouse model was created. The double mutant mice displayed a more profound phenotype of growth retardation and hydrocephalus compared to mice nullizygous for SPAG6 and SPAG16L alone. The double mutant mice died younger, and mortality was significantly higher than in single mutant mice. In addition, the double mutant mice demonstrated pneumonia and its complications, including hemorrhage, edema, and atelectasis, phenotypes not observed in mice nullizygous for mutations in the individual genes. No other cilia-related phenotypic change was detected in double mutant mice including lateralization defects. The ultrastructure of cilia in both the brain and lung of the double mutant mice appeared normal. This model of combined SPAG6 and SPAG16L deficiency provides a new platform to study primary ciliary dyskinesia. The findings also demonstrate that SPAG6 and SPAG16L have related roles in controlling the function of cilia in the brain and lung.

Accelerated mortality from hydrocephalus and pneumonia in mice with a combined deficiency of SPAG6 and SPAG16L reveals a functional interrelationship between the two central apparatus proteins. Publishing Authors By Initials

Z Zhang,W Tang,R Zhou,X Shen,Z Wei,AM Patel,JT Povlishock,J Bennett,JF Strauss,

For similar respiratory tract diseases: lung diseases: pneumonia research abstracts see: respiratory tract diseases: lung diseases: pneumonia research

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Accelerated mortality from hydrocephalus and pneumonia in mice with a combined deficiency of SPAG6 and SPAG16L reveals a functional interrelationship between the two central apparatus proteins. Journal Published:

PUBLICATION TYPE: Research Support, Non-U.S. Gov

Journal: Cell motility and the cytoskeleton

VOLUME: 64

Page Numbers: 360-76

Journal Abbreviation:

ISSN: 0886-1544

DAY: 3

MONTH: May

YEAR: 2007

Accelerated mortality from hydrocephalus and pneumonia in mice with a combined deficiency of SPAG6 and SPAG16L reveals a functional interrelationship between the two central apparatus proteins. Information

Number of References:

LANGUAGE: eng

NlmUniqueID: 8605339

Accelerated mortality from hydrocephalus and pneumonia in mice with a combined deficiency of SPAG6 and SPAG16L reveals a functional interrelationship between the two central apparatus proteins. Keywords Mesh Terms:

KEYWORDS: Pneumonia

MESH TERMS: mortality

Chemical & Substance for Abstract: Accelerated mortality from hydrocephalus and pneumonia in mice with a combined deficiency of SPAG6 and SPAG16L reveals a functional interrelationship between the two central apparatus proteins. Information

Substance Name: SPAG6 protein, mouse

Registry Number: 0

Grant and Affiliation Information for Accelerated mortality from hydrocephalus and pneumonia in mice with a combined deficiency of SPAG6 and SPAG16L reveals a functional interrelationship between the two central apparatus proteins.

AFFILIATION: Department of Obstetrics and Gynecology, Virginia Commonwealth University, Richmond, Virginia 23298, USA. zzhang4@vcu.edu

Country: United States

AGENCY: United States AHRQ

GRANT: HS06724

ACRONYM: HS

MEDLINETA: Cell Motil Cytoskeleton

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