نتایج جستجو برای: imerslund

تعداد نتایج: 58  

2014
J.C. Fyfe S.L. Hemker P.J. Venta B. Stebbing U. Giger

BACKGROUND Selective intestinal cobalamin malabsorption with mild proteinuria (Imerslund-Gräsbeck syndrome; I-GS), is an autosomal recessive disorder of dogs caused by mutations in AMN or CUBN that disrupt cubam function and which can present as a medical emergency. OBJECTIVES To describe the clinical, metabolic, and genetic bases of I-GS in Beagles. ANIMALS Four cobalamin-deficient and 43 ...

2005
Qianchuan He Mette Madsen Adam Kilkenney Brittany Gregory Erik I. Christensen Henrik Vorum Peter Højrup Alejandro A. Schäffer Ewen F. Kirkness Stephan M. Tanner Albert de la Chapelle Urs Giger Søren K. Moestrup John C. Fyfe

Amnionless (AMN) and cubilin gene products appear to be essential functional subunits of an endocytic receptor called cubam. Mutation of either gene causes autosomal recessive Imerslund-Gräsbeck syndrome (I-GS, OMIM no. 261100) in humans, a disorder characterized by selective intestinal malabsorption of cobalamin (vitamin B12) and urinary loss of several specific low-molecular-weight proteins. ...

Journal: :Blood 1998
R Kozyraki M Kristiansen A Silahtaroglu C Hansen C Jacobsen N Tommerup P J Verroust S K Moestrup

Uptake of vitamin B12 (cyanocobalamin) is facilitated by the cobalamin-binder gastric intrinsic factor (IF), which recognizes a 460-kD receptor, cubilin, present in the epithelium of intestine and kidney. Surface plasmon resonance analysis of ligand-affinity-purified human cubilin demonstrated a high-affinity calcium- and cobalamin-dependent binding of IF-cobalamin. Complete cDNA cloning of the...

2013
Marta Owczarek-Lipska Vidhya Jagannathan Cord Drögemüller Sabina Lutz Barbara Glanemann Tosso Leeb Peter H. Kook

Imerslund-Gräsbeck syndrome (IGS) or selective cobalamin malabsorption has been described in humans and dogs. IGS occurs in Border Collies and is inherited as a monogenic autosomal recessive trait in this breed. Using 7 IGS cases and 7 non-affected controls we mapped the causative mutation by genome-wide association and homozygosity mapping to a 3.53 Mb interval on chromosome 2. We re-sequenced...

Journal: :Blood 2005
Qianchuan He Mette Madsen Adam Kilkenney Brittany Gregory Erik I Christensen Henrik Vorum Peter Højrup Alejandro A Schäffer Ewen F Kirkness Stephan M Tanner Albert de la Chapelle Urs Giger Søren K Moestrup John C Fyfe

Amnionless (AMN) and cubilin gene products appear to be essential functional subunits of an endocytic receptor called cubam. Mutation of either gene causes autosomal recessive Imerslund-Gräsbeck syndrome (I-GS, OMIM no. 261100) in humans, a disorder characterized by selective intestinal malabsorption of cobalamin (vitamin B12) and urinary loss of several specific low-molecular-weight proteins. ...

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