macTT
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A 2 year old boy has brittle dipigmented hair, cerebral atrophy, demylinateion, motor delay, and mental retardation. Lysyl oxidase activity is decreased. The function of which of the following subst. is decreased?
A) Albumin
B) Ceruloplasmin
C) Chondritin sulfate
D) Collagen
Even though D is the obvious answer......I was pondering if it is possible to be B as well. Because I thought lysyl oxidase requries Cu2+ as the cofactor. And since ceruloplasimin is involved in copper storage /transport, a deficient in the protein will cause problem with lysyl oxidase activity. Correct me if I am wrong. Thanks a million.
JACK
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| MRMAVERICK
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ME TOOOOOOOO
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what does it have to do with ceruloplasmin. in my knowlodge only 2 things are related to Cu. wilsons ds and menkes ds.
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saif
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actually it is Menkes disease. cuz the brittle hair and motor delay explains all.
Pathophysiology: Copper is a trace metal in many essential enzyme systems, including cytochrome C oxidase, superoxide dismutase, lysyl oxidase, tyrosinase, ascorbic acid oxidase, ceruloplasmin, and dopamine beta hydroxylase. The deficiency or impaired function of these enzyme systems is thought to be responsible for the clinical findings of Menkes disease. The Menkes gene is located on the long arm of the X chromosome at Xq13.3, and the gene product is a 1500–amino acid P-type adenosine triphosphatase (ATPase) that has 17 domains—6 copper binding, 8 transmembrane, a phosphatase, a phosphorylation, and an ATP binding.
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saif
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http://www.emedicine.com/neuro/topic569.htm
u will get everything here
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saif
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| macTT
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So what is the correct answer? Hehe
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ceruloplasmin, B. menkes disease. it didn't came in my mind in the first place. don't go for the obvious. 
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saif
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