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A 5-day-old neonate presents with poor feeding, progressive encephalopathy, and a sweet caramel-like odour to the urine. Plasma amino acid analysis shows markedly elevated leucine, isoleucine, and valine. The newborn screening card is flagged. The relevant catabolic pathway is shown (Image 1), depicting transamination of branched-chain amino acids (leucine, isoleucine, valine) to their corresponding branched-chain α-keto acids, followed by the shared oxidative decarboxylation step (marked with a bold red X) catalysed by the branched-chain α-keto acid dehydrogenase (BCKDH) complex, which converts the α-keto acids to their respective acyl-CoA products. Which coenzyme is directly required by the enzymatic complex blocked at the step indicated in the image?

Which of the following amino acids is not coded by a standard triplet codon?
Cathelicidins are rich in which of the following amino acids?
The conversion of an optically pure isomer (enantiomer) into a mixture of equal amounts of both dextro and levo forms is called as?
What are amphipathic helices?
Amino Acids: Structure and Properties
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Peptide Bond Formation
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Primary Structure of Proteins
Practice Questions
Secondary Structure of Proteins
Practice Questions
Tertiary and Quaternary Structures
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Protein Folding and Chaperones
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Protein Domains and Motifs
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Structure-Function Relationships
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Hemoglobin and Myoglobin
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Collagen and Elastin
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Albumin and Plasma Proteins
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Post-Translational Modifications
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