Table 3.

Key results of comparing the transcripts of skeletal muscle in ALS and DMD to those in healthy: METB model family

Glycolytic
ALS
Up	UGP2, SOD1, PGK1, AK1, CKMT2, CS, GYS1
Down	CKM, GAPDH, ALDOA, ENO3, PYGM
DMD
Up	AK1
Down	CKM, GAPDH, ALDOA, ENO3, PYGM
ALS	Downregulated genes involved in glycolysis and glycogenolysis are consistent with lower use of fast isoforms in the EA and MECH models, and a degree of denervation. Upregulated genes involved in glycogenesis suggest more glycogen storage
DMD	Downregulated genes involved in glycolysis and glycogenolysis suggest limited or slower muscle energy usage
Oxidative phase 1
ALS
Up	Nearly all
Down	ACADS
DMD
Up	None
Down	One-half of them
Oxidative phase 2
ALS
Up	Nearly all
Down	COX7A1, UCP1, UCP3
DMD
Up	None
Down	COX7A1, COX5A, COX6A2, ATP5H, UCP1, UCP3
ALS	Upregulation of nearly all genes in oxidative subfamilies is consistent with elevated ATP production in mitochondria. However, dramatic downregulation of COX7A1 suggests a mitochondrial transformation process from skeletal-specific lattice to a more general type of mitochondria
DMD	Downregulation of nearly all genes in oxidative subfamilies (e.g., COX genes, PGC-1α) is consistent with less ATP production and decreased volume in mitochondria
Transporter
ALS
Up	VDAC1, VDAC2, VDAC3, SLC25A11, SLC25A12, SLC25A5, SLC25A6, SLC38A2
Down	MB, HBA1/HBA2, HBB
DMD
Up	None
Down	SLC25A4, SLC2A4
ALS	Strong downregulation of MB and HB indicates diminished oxygen transportation and storage capacity, consistent with muscle disuse. Yet upregulation of VDAC for the outer membrane and SLC25A (mitochondria adenine nucleotide translocator, ANT) for the inner membrane suggests a degree of hypermetabolism
DMD	Downregulation of SLC2A4 (GLUT-4) is consistent with restrained glucose transportation and of SLC25A4 (ANT) restrained mitochondrial ATP production, both consistent with diminished muscle usage

METB, metabolic and bioenergetics. See legend for Table 1.