Mass Spectrometry-Based Metabolomic and Proteomic Strategies in Organic Acidemias

Table 1

Multisystem involvement in OAs.

	Hearth	Skeletal muscle	Liver	Pancreas	Kidney	Brain
MSUD	+	+	+	+		++
PA	+	+	+	+		++
MMA	+	+	+	+	+	++
IVA	+	+	+	+		+
GA I	+	+	+	+		+
β-KTD	+	+	+	+		+
HMG-CoA lyase D	+	+	+	+		+