Table 2.

Activities of acyl-CoA dehydrogenases and related fatty acid oxidation enzymes in homogenates of liver and skeletal muscle (all activities μmol/min/g wet weight tissue)

Enzyme	Tissue	Control	Patient (% control mean)
SCAD (C4)	SM	0.94±0.36 (0.44-1.95)	0.44 (46)
	L	4.6±2.95 (2.48-8.92)	2.16 (47)
MCAD (C8)	SM	0.88±0.22 (0.61-1.31)	0.69 (78)
	L	2.15±0.38 (1.87-2.7)	2.55 (119)
LCAD (C16)	SM	1.22±0.44 (0.67-2.29)	0.58 (47)
	L	2.52±0.94 (1.46-3.64)	1.26 (50)
3-Hydroxy-ACD (C4)	SM	18.51 ±8.25 (6.0-37.45)	15.62 (84)
	L	17.31 ±5.51 (9.75-24.8)	20.54 (119)
Ketothiolase (C4)	SM	9.61 ±3.48 (4.6-16.8)	0.5 (5)
	L	24.95±2.46 (20.95-27.35)	10.18 (41)
CS	SM	15.56±3.87 (11.35-24.2)	6.23 (40)
	L	7.03±2.53 (5.05-11.85)	6.12 (87)

SCAD=short chain acyl-CoA dehydrogenase;

MCAD=medium chain acyl-CoA dehydrogenase;

LCAD=long chain acyl-CoA dehydrogenase;

3-hydroxy-ACD=3-hydroxyacyl-CoA dehydrogenase;

ketothiolase=short chain beta-ketothiolase;

SM=skeletal muscle;

L=liver; values for control represent mean±SD with range in parenthesis (n=25 for skeletal muscle, n=8 for liver).