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Scholar Results 1 - 10 of about 101 related to Izumi: Dominant negative pathogenesis by mutant proinsulin in the Akita diabetic mouse. (0.09 sec) 

Dominant negative pathogenesis by mutant proinsulin in the Akita diabetic mouse


T Izumi, H Yokota-Hashimoto, S Zhao, J Wang … - Diabetes, 2003 - Am Diabetes Assoc
Autosomal dominant diabetes in the Akita mouse is caused by mutation of the
insulin 2 gene, whose product replaces a cysteine residue that is engaged in the
formation of an intramolecular disulfide bond. These heterozygous mice ...
Cited by 48 - Related articles - BL Direct - All 5 versions

Proteotoxicity in the endoplasmic reticulum: lessons from the Akita diabetic mouse

- nih.gov
D Ron - Journal of Clinical Investigation, 2002 - Am Soc Clin Investig
Mutations that impair polypeptide folding commonly result in an unstable and
hypofunctional gene product. Their phenotype reflects this loss of function, and
the resulting genetic disorder is usually transmitted as a recessive trait. ...
Cited by 61 - Related articles - BL Direct - All 14 versions

A novel locus, Mody4, distal to D7Mit189 on chromosome 7 determines early-onset NIDDM …


M Yoshioka, T Kayo, T Ikeda, A Koizumi - Diabetes, 1997 - Am Diabetes Assoc
In this article, we report on a nonobese C57BL/6 (B6) mouse model of NIDDM named
Akita mouse, characterized by early age onset and autosomal dominant mode of
inheritance. At 7 weeks of age, the mean morning blood glucose levels ...
Cited by 99 - Related articles - BL Direct - All 3 versions

A mutation in the insulin 2 gene induces diabetes with severe pancreatic β-cell dysfunction …

- nih.gov
J Wang, T Takeuchi, S Tanaka, SK Kubo, T … - Journal of Clinical Investigation, 1999 - Am Soc Clin Investig
The mouse autosomal dominant mutation Mody develops hyperglycemia with notable
pancreatic β-cell dysfunction. This study demonstrates that one of the alleles
of the gene for insulin 2 in Mody mice encodes a protein product that ...
Cited by 135 - Related articles - BL Direct - All 10 versions

Proinsulin lacking the A7-B7 disulfide bond, Ins2Akita, tends to aggregate due to the …


T Yoshinaga, K Nakatome, J Nozaki, M Naitoh … - Biological Chemistry, 2005 - reference-global.com
1 Department of Health and Environmental Sciences, Kyoto University Graduate
School of Medicine, Kyoto 606-8501, Japan 2 Department of Molecular and Cellular
Biology, Institute for Frontier Medical Sciences, Kyoto University, Kyoto ...
Cited by 9 - Related articles - BL Direct - All 6 versions

Misfolded proinsulin accumulates in expanded pre-Golgi intermediates and endoplasmic …

- fasebj.org
C Zuber, JY Fan, B Guhl, J Roth - The FASEB Journal, 2004 - FASEB
Figure 1. Fine structural details of ß cells of islets of Langerhans from
control and Akita mice. A) In control mice ß cells, narrow cisternae of rough
endoplasmic reticulum (RER) are continuous (arrowheads) with dilated ...
Cited by 24 - Related articles - BL Direct - All 5 versions

Proinsulin disulfide maturation and misfolding in the endoplasmic reticulum

- nih.gov [PDF] 
M Liu, Y Li, D Cavener, P Arvan - Journal of Biological Chemistry, 2005 - ASBMB
Upon nonreducing Tris-Tricine-urea-SDS-PAGE, newly synthesized proinsulin from
pancreatic islets of normal rodents forms a band fast mobility representing the
native disulfide isomer, which is efficiently secreted. In addition at ...
Cited by 14 - Related articles - All 6 versions

Familial hyperproinsulinaemia due to a mutation substituting histidine for arginine at position …


M Collinet, M Berthelon, P Bénit, K Laborde, … - European journal of pediatrics, 1998 - Springer
Abstract Familial hyperproinsulinaemia is a rare genetic disorder characterized
by point mutations in the insulin gene which impair the conversion of proinsulin
to insulin. We report here three members of a two-generation Caucasian ...
Cited by 5 - Related articles - BL Direct - All 3 versions

Longitudinal evaluation of memory performance and peripheral neuropathy in the Ins2C96Y …


C Choeiri, K Hewitt, J Durkin, CJ Simard, JM … - Behavioural Brain Research, 2005 - Elsevier
Ins2 C96Y Akita mice represent a model of spontaneous early-onset diabetes
mellitus, expressing a mutant non-functional isoform of insulin. These mice are
characterized by a reduced number of pancreatic β cells resulting in ...
Cited by 8 - Related articles - All 2 versions

Diabetes in very young children and mutations in the insulin-secreting cell potassium …


I Flechtner, M Vaxillaire, H Cav├®, R … - Endocrine development, 2007 - content.karger.com
Shield JPH, Scharfmann R (eds): Development of the Pancreas and Neonatal
Diabetes. Endocr Dev. Basel, Karger, 2007, vol 12, pp 86–98 ... Abstract
ATP-sensitive potassium (K ATP ) channels regulate the flux of K + ions ...
Cited by 8 - Related articles - BL Direct - All 4 versions


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