Proteins > Delta-1-pyrroline-5-carboxylate dehydrogenase (ALDH4A1)
Delta-1-pyrroline-5-carboxylate dehydrogenase (ALDH4A1)
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UniProt Number:
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P30038
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Alternate Names:
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aldehyde dehydrogenase family 4 member A1, P5C dehydrogenase, ALDH4, P5CDH
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Structure and Function:
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ALDH4A1 belongs to the aldehyde dehydrogenase family of proteins. This enzyme, present in the mitochondrial matrix, converts pyrroline-5-carboxylate to glutamate (the second step of the proline degradation pathway) and is an NAD-dependent dehydrogenase.
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Disease Associations:
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Deficiency of this enzyme is associated with type II hyperprolinemia, an autosomal recessive disorder characterized by accumulation of delta-1-pyrroline-5-carboxylate (P5C) and proline. [MIM: 239510]
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Protein Quantity Assays
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