TY  - CHAP
M1  - Book, Section
TI  - Mitochondrial Fatty Acid Oxidation Defects
A1  - Vockley, Jerry
A1  - Longo, Nicola
A1  - Andresen, Brage S.
A1  - Bennett, Michael J.
A2  - Sarafoglou, Kyriakie
A2  - Hoffmann, Georg F.
A2  - Roth, Karl S.
PY  - 2017
T2  - Pediatric Endocrinology and Inborn Errors of Metabolism, 2e
AB  - Mitochondria  can  utilize  carbohydrate,  protein,  or  fat  as  a  source  of  energy.  Pyruvate,  derived  from  glucose  or  amino  acids,  is  transported  through  the  mitochondrial  inner  membrane  using  a  specific  transporter,1  decarboxylated  to  acetyl-coenzyme  A  (CoA),  and  enters  the  citric  acid  cycle  producing  reducing  equivalents  in  the  form  of  1,5-dihydroflavin  adenine  dinucleotide  (FADH2)  and  nicotinamide  adenine  dinucleotide  (NADH).  These  reducing  equivalents  are  transported  down  the  electron  transport  chain  of  inner  mitochondrial  membrane  complexes,  ultimately  generating  adenosine  triphosphate  (ATP)  and  consuming  oxygen.  Similarly,  activated  fatty  acids  (acyl-CoAs)  entering  the  fatty  acid  β-oxidation  (FAO)  spiral  generate  reducing  equivalents  in  the  form  of  NADH  and  FADH2,  which  also  pass  down  the  oxidative  phosphorylation  respiratory  chain  complexes  to  generate  ATP.  
SN  - 
PB  - McGraw-Hill Education
CY  - New York, NY
Y2  - 2024/04/18
UR  - accesspediatrics.mhmedical.com/content.aspx?aid=1140315409
ER  -