-
1
-
-
0842287450
-
Mitochondrial β-oxidation
-
Bartlett K, Eaton S. Mitochondrial β-oxidation. Eur J Biochem. 2004;271(3):462-469.
-
(2004)
Eur J Biochem.
, vol.271
, Issue.3
, pp. 462-469
-
-
Bartlett, K.1
Eaton, S.2
-
2
-
-
0032969056
-
Disorders of mitochondrial fatty acyl-CoA β-oxidation
-
Wanders RJ, Vreken P, den Boer ME, Wijburg FA, van Gennip AH, Ijlst L. Disorders of mitochondrial fatty acyl-CoA β-oxidation. J Inherit Metab Dis. 1999;22(4):442-487.
-
(1999)
J Inherit Metab Dis.
, vol.22
, Issue.4
, pp. 442-487
-
-
Wanders, R.J.1
Vreken, P.2
Den Boer, M.E.3
Wijburg, F.A.4
Van Gennip, A.H.5
Ijlst, L.6
-
4
-
-
77957554125
-
Pathophysiology of fatty acid oxidation disorders
-
Bennett MJ. Pathophysiology of fatty acid oxidation disorders. J Inherit Metab Dis. 2010;33(5):533-537.
-
(2010)
J Inherit Metab Dis.
, vol.33
, Issue.5
, pp. 533-537
-
-
Bennett, M.J.1
-
5
-
-
0015919670
-
L-3-hydroxyacyl coenzyme A dehydrogenase from pig heart muscle. I. Purification and properties
-
Noyes BE, Bradshaw RA. L-3-Hydroxyacyl coenzyme A dehydrogenase from pig heart muscle. I. Purification and properties. J Biol Chem. 1973;248(9):3052- 3059.
-
(1973)
J Biol Chem.
, vol.248
, Issue.9
, pp. 3052-3059
-
-
Noyes, B.E.1
Bradshaw, R.A.2
-
6
-
-
0024382885
-
Assay of L-3-hydroxyacyl-coenzyme A dehydrogenase with substrates of different chain lengths
-
He XY, Yang SY, Schulz H. Assay of L-3-hydroxyacyl-coenzyme A dehydrogenase with substrates of different chain lengths. Anal Biochem. 1989;180(1):105-109.
-
(1989)
Anal Biochem.
, vol.180
, Issue.1
, pp. 105-109
-
-
He, X.Y.1
Yang, S.Y.2
Schulz, H.3
-
7
-
-
0030601110
-
Human short-chain L-3-hydroxyacyl-CoA dehydrogenase: Cloning and characterization of the coding sequence
-
Vredendaal PJ, van den Berg IE, Malingré HE, Stroobants AK, Olde Weghuis DE, Berger R. Human short-chain L-3-hydroxyacyl-CoA dehydrogenase: cloning and characterization of the coding sequence. Biochem Biophys Res Commun. 1996;223(3):718-723.
-
(1996)
Biochem Biophys Res Commun.
, vol.223
, Issue.3
, pp. 718-723
-
-
Vredendaal, P.J.1
Van Den-Berg, I.E.2
Malingré, H.E.3
Stroobants, A.K.4
Olde Weghuis, D.E.5
Berger, R.6
-
8
-
-
0017746899
-
Hydroxyacyl CoA dehydrogenase, an enzyme important in fat metabolism in different cell types in the islets of Langerhans
-
Agren A, Borg K, Brolin SE, Carlman J, Lundqvist G. Hydroxyacyl CoA dehydrogenase, an enzyme important in fat metabolism in different cell types in the islets of Langerhans. Diabetes Metab. 1977; 3(3):169-172.
-
(1977)
Diabetes Metab.
, vol.3
, Issue.3
, pp. 169-172
-
-
Agren, A.1
Borg, K.2
Brolin, S.E.3
Carlman, J.4
Lundqvist, G.5
-
9
-
-
34547095291
-
Specificity in β cell expression of L-3-hydroxyacyl-CoA dehydrogenase, short chain, and potential role in down-regulating insulin release
-
Martens GA, Vervoort A, Van de CM, et al. Specificity in β cell expression of L-3-hydroxyacyl-CoA dehydrogenase, short chain, and potential role in down-regulating insulin release. J Biol Chem. 2007;282(29):21134-21144.
-
(2007)
J Biol Chem.
, vol.282
, Issue.29
, pp. 21134-21144
-
-
Martens, G.A.1
Vervoort, A.2
Van De, C.M.3
-
10
-
-
85047691646
-
Foxa2 regulates multiple pathways of insulin secretion
-
Lantz KA, Vatamaniuk MZ, Brestelli JE, Friedman JR, Matschinsky FM, Kaestner KH. Foxa2 regulates multiple pathways of insulin secretion. J Clin Invest 2004;114(4):512-520.
-
(2004)
J Clin Invest
, vol.114
, Issue.4
, pp. 512-520
-
-
Lantz, K.A.1
Vatamaniuk, M.Z.2
Brestelli, J.E.3
Friedman, J.R.4
Matschinsky, F.M.5
Kaestner, K.H.6
-
11
-
-
0035394988
-
Tissue-specific deletion of Foxa2 in pancreatic β cells results in hyperinsulinemic hypoglycemia
-
Sund NJ, Vatamaniuk MZ, Casey M, et al. Tissue-specific deletion of Foxa2 in pancreatic β cells results in hyperinsulinemic hypoglycemia. Genes Dev. 2001;15(13):1706-1715.
-
(2001)
Genes Dev.
, vol.15
, Issue.13
, pp. 1706-1715
-
-
Sund, N.J.1
Vatamaniuk, M.Z.2
Casey, M.3
-
12
-
-
0034902277
-
Hyperinsulinism in short-chain L-3-hydroxyacyl-CoA dehydrogenase deficiency reveals the importance of β-oxidation in insulin secretion
-
Clayton PT, Eaton S, Aynsley-Green A, et al. Hyperinsulinism in short-chain L-3-hydroxyacyl-CoA dehydrogenase deficiency reveals the importance of β-oxidation in insulin secretion. J Clin Invest. 2001;108(3):457-465.
-
(2001)
J Clin Invest.
, vol.108
, Issue.3
, pp. 457-465
-
-
Clayton, P.T.1
Eaton, S.2
Aynsley-Green, A.3
-
13
-
-
0347990591
-
Familial hyperinsulinemic hypoglycemia caused by a defect in the SCHAD enzyme of mitochondrial fatty acid oxidation
-
Molven A, Matre GE, Duran M, et al. Familial hyperinsulinemic hypoglycemia caused by a defect in the SCHAD enzyme of mitochondrial fatty acid oxidation. Diabetes. 2004;53(1):221-227.
-
(2004)
Diabetes.
, vol.53
, Issue.1
, pp. 221-227
-
-
Molven, A.1
Matre, G.E.2
Duran, M.3
-
14
-
-
20944434799
-
Hyperinsulinism of infancy associated with a novel splice site mutation in the SCHAD gene
-
Hussain K, Clayton PT, Krywawych S, et al. Hyperinsulinism of infancy associated with a novel splice site mutation in the SCHAD gene. J Pediatr. 2005;146(5):706-708.
-
(2005)
J Pediatr.
, vol.146
, Issue.5
, pp. 706-708
-
-
Hussain, K.1
Clayton, P.T.2
Krywawych, S.3
-
15
-
-
67650221414
-
3-Hydroxyacyl-coenzyme A dehydrogenase deficiency and hyperinsulinemic hypoglycemia: Characterization of a novel mutation and severe dietary protein sensitivity
-
Kapoor RR, James C, Flanagan SE, Ellard S, Eaton S, Hussain K. 3-Hydroxyacyl-coenzyme A dehydrogenase deficiency and hyperinsulinemic hypoglycemia: characterization of a novel mutation and severe dietary protein sensitivity. J Clin Endocrinol Metab. 2009; 94(7):2221-2225.
-
(2009)
J Clin Endocrinol Metab.
, vol.94
, Issue.7
, pp. 2221-2225
-
-
Kapoor, R.R.1
James, C.2
Flanagan, S.E.3
Ellard, S.4
Eaton, S.5
Hussain, K.6
-
16
-
-
66149153000
-
Identification of a diffuse form of hyperinsulinemic hypoglycemia by 18-fluoro-L-3,4 dihydroxyphenylalanine positron emission tomography/CT in a patient carrying a novel mutation of the HADH gene
-
Di CS, Gessi A, Pepe G, et al. Identification of a diffuse form of hyperinsulinemic hypoglycemia by 18-fluoro-L-3,4 dihydroxyphenylalanine positron emission tomography/CT in a patient carrying a novel mutation of the HADH gene. Eur J Endocrinol. 2009; 160(6):1019-1023.
-
(2009)
Eur J Endocrinol.
, vol.160
, Issue.6
, pp. 1019-1023
-
-
Di, C.S.1
Gessi, A.2
Pepe, G.3
-
17
-
-
79959771790
-
Short-chain 3-hydroxyacyl-CoA dehydrogenase deficiency: The clinical relevance of an early diagnosis and report of four new cases
-
Martins E, Cardoso ML, Rodrigues E, et al. Short-chain 3-hydroxyacyl-CoA dehydrogenase deficiency: the clinical relevance of an early diagnosis and report of four new cases. J Inherit Metab Dis. 2011;34(3):835-842.
-
(2011)
J Inherit Metab Dis.
, vol.34
, Issue.3
, pp. 835-842
-
-
Martins, E.1
Cardoso, M.L.2
Rodrigues, E.3
-
18
-
-
0346788907
-
Short-chain 3-hydroxyacyl-CoA dehydrogenase deficiency associated with hyperinsulinism: A novel glucose-fatty acid cycle?
-
Eaton S, Chatziandreou I, Krywawych S, Pen S, Clayton PT, Hussain K. Short-chain 3-hydroxyacyl-CoA dehydrogenase deficiency associated with hyperinsulinism: a novel glucose-fatty acid cycle? Biochem Soc Trans. 2003;31(Pt 6):1137-1139.
-
(2003)
Biochem Soc Trans.
, vol.31
, Issue.PART 6
, pp. 1137-1139
-
-
Eaton, S.1
Chatziandreou, I.2
Krywawych, S.3
Pen, S.4
Clayton, P.T.5
Hussain, K.6
-
19
-
-
0000421978
-
Familial hypoglycemia precipitated by amino acids
-
Cochrane WA, Payne WW, Simpkiss MJ, Woolf LI. Familial hypoglycemia precipitated by amino acids. J Clin Invest. 1956;35(4): 411-422.
-
(1956)
J Clin Invest.
, vol.35
, Issue.4
, pp. 411-422
-
-
Cochrane, W.A.1
Payne, W.W.2
Simpkiss, M.J.3
Woolf, L.I.4
-
20
-
-
0023695196
-
Regulation of insulin release by factors that also modify glutamate dehydrogenase
-
Fahien LA, MacDonald MJ, Kmiotek EH, Mertz RJ, Fahien CM. Regulation of insulin release by factors that also modify glutamate dehydrogenase. J Biol Chem. 1988;263(27):13610-13614.
-
(1988)
J Biol Chem.
, vol.263
, Issue.27
, pp. 13610-13614
-
-
Fahien, L.A.1
MacDonald, M.J.2
Kmiotek, E.H.3
Mertz, R.J.4
Fahien, C.M.5
-
21
-
-
33745955624
-
Protein-sensitive hypoglycemia without leucine sensitivity in hyperinsulinism caused by K(ATP) channel mutations
-
Fourtner SH, Stanley CA, Kelly A. Protein-sensitive hypoglycemia without leucine sensitivity in hyperinsulinism caused by K(ATP) channel mutations. J Pediatr. 2006;149(1):47-52.
-
(2006)
J Pediatr.
, vol.149
, Issue.1
, pp. 47-52
-
-
Fourtner, S.H.1
Stanley, C.A.2
Kelly, A.3
-
22
-
-
77957760755
-
Mechanism of hyperinsulinism in short-chain 3-hydroxyacyl-CoA dehydrogenase deficiency involves activation of glutamate dehydrogenase
-
Li C, Chen P, Palladino A, et al. Mechanism of hyperinsulinism in short-chain 3-hydroxyacyl-CoA dehydrogenase deficiency involves activation of glutamate dehydrogenase. J Biol Chem. 2010;285(41): 31806-31818.
-
(2010)
J Biol Chem.
, vol.285
, Issue.41
, pp. 31806-31818
-
-
Li, C.1
Chen, P.2
Palladino, A.3
-
23
-
-
84860809004
-
Leucine-sensitive hyperinsulinaemic hypoglycaemia in patients with loss of function mutations in 3-hydroxyacyl-CoA dehydrogenase
-
Heslegrave AJ, Kapoor RR, Eaton S, et al. Leucine-sensitive hyperinsulinaemic hypoglycaemia in patients with loss of function mutations in 3-hydroxyacyl-CoA dehydrogenase. Orphanet J Rare Dis. 2012;7(1):25.
-
(2012)
Orphanet J Rare Dis.
, vol.7
, Issue.1
, pp. 25
-
-
Heslegrave, A.J.1
Kapoor, R.R.2
Eaton, S.3
-
24
-
-
39149123797
-
Role of short-chain hydroxyacyl CoA dehydrogenases in SCHAD deficiency
-
Filling C, Keller B, Hirschberg D, et al. Role of short-chain hydroxyacyl CoA dehydrogenases in SCHAD deficiency. Biochem Biophys Res Commun. 2008;368(1):6-11.
-
(2008)
Biochem Biophys Res Commun.
, vol.368
, Issue.1
, pp. 6-11
-
-
Filling, C.1
Keller, B.2
Hirschberg, D.3
-
25
-
-
77952626170
-
Systemic activation of glutamate dehydrogenase increases renal ammoniagenesis: Implications for the hyperinsulinism/hyperammonemia syndrome
-
Treberg JR, Clow KA, Greene KA, Brosnan ME, Brosnan JT. Systemic activation of glutamate dehydrogenase increases renal ammoniagenesis: implications for the hyperinsulinism/hyperammonemia syndrome. Am J Physiol Endocrinol Metab. 2010;298(6):E1219-E1225.
-
(2010)
Am J Physiol Endocrinol Metab
, vol.298
, Issue.6
-
-
Treberg, J.R.1
Clow, K.A.2
Greene, K.A.3
Brosnan, M.E.4
Brosnan, J.T.5
-
26
-
-
80052271742
-
The structure and allosteric regulation of glutamate dehydrogenase
-
Li M, Li C, Allen A, Stanley CA, Smith TJ. The structure and allosteric regulation of glutamate dehydrogenase. Neurochem Int. 2011;59(4):445-455.
-
(2011)
Neurochem Int.
, vol.59
, Issue.4
, pp. 445-455
-
-
Li, M.1
Li, C.2
Allen, A.3
Stanley, C.A.4
Smith, T.J.5
-
27
-
-
84857804808
-
The structure and allosteric regulation of mammalian glutamate dehydrogenase
-
Li M, Li C, Allen A, Stanley CA, Smith TJ. The structure and allosteric regulation of mammalian glutamate dehydrogenase. Arch Biochem Biophys. 2012;519(2):69-80.
-
(2012)
Arch Biochem Biophys.
, vol.519
, Issue.2
, pp. 69-80
-
-
Li, M.1
Li, C.2
Allen, A.3
Stanley, C.A.4
Smith, T.J.5
-
28
-
-
84859506954
-
Short-chain 3-hydroxyacyl-coenzyme A dehydrogenase associates with a protein super-complex integrating multiple metabolic pathways
-
Narayan SB, Master SR, Sireci AN, et al. Short-chain 3-hydroxyacyl- coenzyme A dehydrogenase associates with a protein super-complex integrating multiple metabolic pathways. PLoS One. 2012; 7(4):e35048.
-
(2012)
PLoS One.
, vol.7
, Issue.4
-
-
Narayan, S.B.1
Master, S.R.2
Sireci, A.N.3
|