mani
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why is there dicarboxilic acidemia in MCAD deficiency? :?:
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| santaclara
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dicarboxilic acidemia comes from alternate oxidation of fat in MCAD DEF.
and also it is the marker for MCAD in urine as well.
correct me if wrong.
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| mani
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why would there be alternate oxidation of fat in MCAD def?? in beta oxidation, to free each actyl CoA residue a 4 step cycle is to be repeated and MCAD is needed for 1 of these 4 steps. so in my opinion there shouldnt be any oxidation of fatty acid in MCAD def.
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| santaclara
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u r right. but i think MCAD DEF is associated with a block in hepatic beta oxidation b/c primary etiology is hepatic.
so according to lectures of kapaln what i understand is that (alternative source of oxidation of fat in MCAD DEF.)
may be i m worng.
thanx
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| mani
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any more comments about it?
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| Mars-Aris
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in MCAD--> accumulation of monocarboxylic fatty acids and dicarboxylic organic acids, which are structural analogues of the fatty acids that cannot pass through the MCAD step.
--> These dicarboxylic acids are formed by an alternative metabolic pathway called w-oxidation that attempts, without success, to begin oxidation at the opposite end of the fatty acid. These w-oxidation products appear in urine --> dicarboxyllic aciduria
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| mani
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i get it mars-aris, thanks.
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