alexa Mixed Inbred FVB;B6 Background Strain Attenuates Kidney Disease and Improves Survival of Gnem712t/M712T Mice
ISSN: 2168-9547

Molecular Biology: Open Access
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Research Article

Mixed Inbred FVB;B6 Background Strain Attenuates Kidney Disease and Improves Survival of Gnem712t/M712T Mice

Yadira Valles-Ayoub1,2, Arman Haghighatgoo1,2, Chai Saechao1, Zeshan Khokher1,2, Christopher Creencia3, Oscar Scremin2,4, Gregory Lawson, DVM2,5, Babak Darvish2 and Daniel Darvish1,2*

1HIBM Research Group, Reseda, CA

2VA Greater Los Angeles (VA-GLA/UCLA), Los Angeles, CA

3Tissue Procurement Core Laboratory, Department of Pathology and Laboratory Medicine, UCLA School of Medicine, Los Angeles, CA

4Molecular, Cellular & Integrative Physiology IDP, UCLA, Los Angeles, CA

5Division of Laboratory Animal Medicine, UCLA, Los Angeles, CA

*Corresponding Author:
Daniel Darvish, MD
HIBM Research Group, 18341 Sherman Way #201A
Reseda, CA 91335
Tel: 818-789-1033 x 3
Fax: 818-741-3727
E-mail: [email protected]

Received date March 03, 2012; Accepted date March 26, 2012; Published date April 01, 2012

Citation: Valles-Ayoub Y, Haghighatgoo A, Saechao C, Khokher Z, Creencia C, et al. (2012) Mixed Inbred FVB;B6 Background Strain Attenuates Kidney Disease and Improves Survival of Gnem712t/M712T Mice. Molecular Biology 1:104. doi:10.4172/2168-9547.1000104

Copyright: © 2012 Valles-Ayoub Y, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.



Recessive form of Hereditary Inclusion Body Myopathy (HIBM, IBM2, MIM:600737) is an adult onset muscle wasting disorders caused by hypomorphic GNE, the rate-limiting enzyme of sialic acid (Sia) biosynthesis. Unlike human patients, mice bearing the GneM712T/M712T genotype in C57BL/6 background strain suffer severe glomerular hematuria, incomplete podocyte development, and do not survive beyond the first few days of life. We crossed heterozygous mice (GneM712T/+) of B6 strain with FVB strain mice. In mixed inbred FVB;B6 background, the homozygous mice show attenuated glomerular disease and survive longer (mean survival 22±13 weeks, n=26). Paradoxically, the homozygous mice showed increased total Sia levels in serum (2x control), and Neu5Ac:Neu5Gc ratios are slightly shifted towards Neu5Ac in serum and towards Neu5Gc in muscle tissue. Increase in serum Sia levels may be caused by altered glomerular filtration. This paradoxical increase in serum Sia may contribute to Sia pools of muscle, and exert a potential beneficial effect. In summary, the background strain of mouse model can significantly affect the disease phenotype.


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