TABLE 5.

Plasma amino acid concentrations in partial– and null–ornithine transcarbamylase–deficient (partial- and null-OTCD) subjects who received treatment with either sodium phenylbutyrate (10 g · m⁻² · d⁻¹) or phenylbutyrate plus branched-chain amino acid (BCAA) supplementation¹

	Partial-OTCD subjects (n = 7)2		Null-OTCD subjects (n = 3)2			P values
Amino acid	Phenylbutyrate	BCAA	Phenylbutyrate	BCAA	Pooled SEM3	TRT	Gen	TRT × Gen
	μmol/L		μmol/L		μmol/L
Leucine	95	117	69	74	15.2	<0.210	<0.205	<0.400
Isoleucine	52	60	34	38	8.2	<0.274	<0.164	<0.714
Valine	164	176	122	110	19.4	<0.996	<0.126	<0.221
Methionine	30	34	18	16	4.5	<0.849	<0.035	<0.593
Cysteine	17	17	25	33	5.1	<0.198	<0.207	<0.217
Phenylalanine	79	74	58	53	4.5	<0.145	<0.009	<0.928
Tyrosine	93	82	49	51	6.5	<0.231	<0.003	<0.077
Lysine	193	178	111	110	18.3	<0.241	<0.032	<0.279
Arginine	64a,b	58b	71a,b	93a	6.2	<0.110	<0.056	<0.008
Ornithine	47	46	62	64	9.7	<0.864	<0.337	<0.780
Citrulline	19	22	121	178	25.8	<0.290	<0.004	<0.349
Glutamate	68	61	107	76	11.7	<0.018	<0.203	<0.129
Glutamine	679	610	424	497	76.2	<0.938	<0.192	<0.023
Aspartate	6	7	4	2	1.5	<0.804	<0.227	<0.306
Asparagine	68	72	46	46	7.8	<0.670	<0.096	<0.697
Ammonia	10	11	36	27	4.9	<0.347	<0.009	<0.299

¹TRT, treatment (phenylbutyrate compared with phenylbutyrate plus BCAA treatments); Gen, genotype (partial-OTCD compared with null-OTCD subjects); TRT × Gen, interaction of main effects. Data were analyzed by using mixed models (fixed effects for TRT, Gen, and TRT × Gen; random effects for subject). Values with different superscript letters differed (Tukey-Kramer–adjusted P < 0.05).

²Values are means.

³On the basis of n = 7.