Metallothionein messenger RNA regulation in the macular mutant mouse: An animal model of Menkes's kinky-hair disease
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چکیده
منابع مشابه
Metallothionein messenger RNA regulation in the mottled mouse and Menkes kinky hair syndrome.
Menkes kinky hair syndrome is an X-linked neurodegenerative disorder, causing tissue-specific increases in copper and metallothionein content. A mouse model is provided by hemizygotes for mutant alleles at the X-linked mottled locus. Herein we test the possibility that the primary defect in both species is in metallothionein gene regulation. We show that metallothionein-I messenger RNA (mRNA) (...
متن کاملCerebral MR of Menkes kinky-hair disease.
Menkes kinky-hair disease (trichopoliodystrophy, steelyhair disease) is an X-linked neurodegenerative disorder that occurs predominantly in males. It was first described by Menkes in 1962 [1]. The characteristic clinical features are steely hair, profound retardation, spastic quadriparesis, seizures, and hypothermia. The disease is caused by an underlying defect of copper metabolism; and the se...
متن کاملMenkes' Kinky Hair disease: new considerations.
Kinky hair disease (KHD) was first described by Menkes et al.(1). Ten years later Danks et al. suggested that the primary defect is in copper transport leading to copper deficiency(2). It is a Xlinked recessive disorder. The gene for KHD is about 200 kb in size and is located on the long arm of X chromosome (Xql3.3). The cDNA has been recently sequenced and is known to code for a protein of 150...
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An eight month old male infant with protein energy malnutrition was admitted in the hospital with the history of repeated attacks of convulsion since four months of age. He was also suffering from frequent attacks of cough and cold since 6 months of age which was marked prior to admission. The infant had fair complexion, sparse fuzzy wooly hair with marked trunkal hypotonia. He had also mental ...
متن کاملMessenger RNA in animal cells.
It is difficult to believe that only 2 years ago, the very existence of messenger RNAs in animal cells was doubtful, and no definitive example of an isolated and purified mRNA could be given. Since that time, the mRNAs for globin (from rabbit, man, mouse, duck, and guinea pig), lens crystallin (chick and cow), ovalbumin, myosin, and silk fibroin have been isolated and their identities confirmed...
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ژورنال
عنوان ژورنال: Okayama Igakkai Zasshi (Journal of Okayama Medical Association)
سال: 1989
ISSN: 0030-1558,1882-4528
DOI: 10.4044/joma1947.101.5-6_495