Branched chain amino acid sufficiency is necessary for proper luteinizing hormone response, testosterone synthesis, and motor coordination

Loss of branched-chain α-keto acid dehydrogenase kinase (BCKDK) increases branched-chain amino acid (BCAA) oxidation by disinhibiting BCKDH. We previously observed that in Bckdk knockout (KO) mice circulating BCAA levels are significantly reduced, negatively affecting neurological function, motor behavior, and male fertility. This work comparatively assessed how systemic loss of Bckdk modulates the transcriptome in testes and skeletal muscle under control diet feeding. Freely-fed adult male C57BL6/J mice and Bckdk KO mice on the C57BL6/J genetic background were euthanized by decapitation at the mid-point of the light cycle and tissues were quickly harvested and frozen in liquid nitrogen. Total RNA was extracted from frozen testes and hind-limb muscle (gastrocnemius and plantaris combined). Mice aged 5-6 months old carrying a mutant Bckdk allele (BckdkM) were backcrossed onto the C57BL/6J background for at least 10 generations and maintained in a conventional facility with commercial rodent diet (5001 Laboratory Rodent Diet; LabDiet) and tap water provided freely. Skeletal muscle and testis tissues were collected from freely-fed male C57BL6/J wild-type mice and BckdkM mice euthanized at the mid-point of the light cycle.