Familial isolated hyperCKaemia associated with a new mutation in the caveolin-3 (CAV-3) gene

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Familial isolated hyperCKaemia associated with a new mutation in the caveolin-3 (CAV-3) gene.

An 18 year old man and his mother both presented with persistent, isolated raised serum creatine kinase (hyperCKaemia) without muscle symptoms. Analysis of caveolin-3 protein expression in muscle biopsy of the propositus showed a reduction in the protein. Genetic analysis revealed a new heterozygous mutation in the caveolin-3 (CAV-3) gene: a C-->T transition at nucleotide position 83 in exon 1 ...

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Novel missense mutation in the caveolin-3 gene in a Belgian family with rippling muscle disease.

Rippling muscle disease (RMD) is a rare muscle disorder characterised by muscle stiffness, exercise induced myalgia, and cramp-like sensations. It is genetically heterogeneous and can be acquired, but most cases show autosomal dominant inheritance due to mutations in the caveolin-3 (CAV3) gene. We report a novel heterozygous missense mutation in CAV3 in a Belgian family with autosomal dominant ...

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Familial partial lipodystrophy type 3: a new mutation on the PPARG gene.

Familial partial lipodystrophy is an autosomal dominant genetic disorder characterized by lipoatrophy of the extremities and gluteal region and lipohypertrophy of the face, neck and/or trunk. It is associated with insulin resistance, hypertriglyceridemia and increased risk of recurrent episodes of pancreatitis. The PPARG mutation forms, called familial partial lipodystrophies type 3, are very r...

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Atrial standstill in a pediatric patient with associated caveolin-3 mutation

Introduction Atrial standstill (AS), a rare arrhythmogenic condition, is defined by (1) the absence of P waves in surface and intracavitary electrocardiograms (ECGs), (2) the absence of A waves in jugular venous pulse and right atrial pressure tracings, (3) the presence of a supraventricular type QRS complex, (4) the immobility of the atria on fluoroscopy, and (5) the inability to stimulate the...

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A novel missense mutation in the caveolin-3 gene in rippling muscle disease.

Rippling muscle disease (RMD) is a benign myopathy with symptoms and signs of muscular hyperirritability. We report a 17-year-old patient who presented with muscular hypertrophy, local mounding on percussion, and a rippling phenomenon. Needle electromyography showed electrical silence during the rippling phenomenon. Muscle protein immunohistochemical analysis showed a partial deficiency of cave...

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ژورنال

عنوان ژورنال: Journal of Neurology, Neurosurgery & Psychiatry

سال: 2002

ISSN: 0022-3050

DOI: 10.1136/jnnp.73.1.65