Obesity & Lipodystrophy - Medical College of Wisconsin

AGPAT2 mutations in CGL, type 1
(TG biosynthetic pathway)
2-Monoacylglycerol
H 2
C
HC
OH
OH
GPAT
H 2
C
HC
O
OH
O
C
R 1
AGPAT2
mutated
in CGL1
AGPAT
H 2
C
HC
O
O
O
C
O
C
R 1
R 2
PP
Acyl-CoA
CoA
H 2
C
HC
O
O
MGAT
O
C
O
C
R 1
R 2
DGAT
H 2
C
HC
O
O
O
C
O
C
O
R 1
R 2
H 2
C
OPO 3
2-
Glycerol-
3-Phosphate
Acyl-
CoA
CoA
H 2
C
OPO 3
2-
1-Acylglycerol-
3-Phosphate
(Lysophosphatidic Acid)
Acyl-
CoA
CoA
H 2
C
OPO 3
2 -
1,2 Diacylglycerol
Phosphate
(Phosphatidic Acid)
H 2
C
O H
H 2 O P i Acyl -
CoA
CoA
H 2
C
1,2 Diacylglycerol Triacylglycerol
O
C
R 3
Phosphatidyl Inositol
Cardiolipin
Phosphatidyl Choline
Phosphatidyl Ethanolamine
Phosphatidyl Serine