William James Craigen, M.D., Ph.D.
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Positions
- Professor
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Molecular and Human Genetics
Baylor College of Medicine
Houston, TX US
- Professor
-
Pediatrics
Baylor College of Medicine
- Professor
-
Program in Translational Biology and Molecular Medicine
Baylor College of Medicine
- Director
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Metabolic Clinic
Baylor College of Medicine
- Medical Director
-
Baylor Genetics
- Member
-
Dan L Duncan Comprehensive Cancer Center
Baylor College of Medicine
Houston, Texas United States
Education
- BS from University Of Texas At Austin
- 01/1981 - Austin, TX United States
- BA from University Of Texas At Austin
- 01/1981 - Austin, TX United States
- PhD from Baylor College Of Medicine
- 01/1987 - Houston, TX United States
- MD from Baylor College Of Medicine
- 01/1988 - Houston, TX United States
- Residency at Baylor College Of Medicine Affiliate Hospitals
- 01/1988 - Houston, Texas United States
- Pediatrics
- Clinical Fellowship at Baylor College Of Medicine Affiliate Hospitals
- 01/1990 - Houston, Texas United States
- Medical Genetics
Certifications
- Clinical Genetics
- American Board of Medical Genetics
- Clinical Biochemical Genetics
- American Board of Medical Genetics
Professional Interests
- Regulation of cellular energy metabolism
- Genetic Disorders and Metabolic Disorders
- Mouse models of metabolic diseases
Professional Statement
Mitochondrial Function: Mitochondria are now recognized to play a variety of important physiologic roles in various processes beyond ATP synthesis, including programmed cell death (apoptosis), retrograde signaling, cellular proliferation and the regulation of intermediary metabolism. One area of interest in the lab is in understanding the role of the mitochondrial outer membrane permeability in the regulation of cellular energy economy, apoptosis and mammalian organ function. Voltage-dependent Anion Channels (VDAC1-3: also known as mitochondrial porins) are a family of mitochondrial outer membrane proteins that conduct small molecules across the outer membrane. VDACs also bind cytosolic kinases such as hexokinase isoforms, and may act to tether other multi-protein complexes to mitochondria. One isoform (VDAC2) functions in suppressing apoptosis by binding the multi-domain pro-apoptotic protein BAK, while other isoforms play roles in glucose metabolism, learning and memory and fertility. Using model organisms we are interested in determining the specific functions of each isoform in biology and relating VDAC function to disease states. These studies involve biochemical, physiologic and genetic experimentation.Human Metabolic Disorders: Despite advances in identifying human metabolic diseases, pathophysiologic mechanisms are poorly understood and specific treatment strategies lacking. Other projects in the laboratory involve studies of metabolic pathways leading to human inherited disorders. Using mutant mice, our current studies are designed to understand the metabolic disturbances that are associated defects in phospholipid and fatty acid metabolism, purine and creatine synthesis and mitochondrial respiratory chain activities. We are interested in defining cell type-specific functions for the enzymes of intermediary metabolism using knockout mice in conjunction with tissue-specific transgene expression.
Websites
Selected Publications
- Craigen WJ, Graham BH, Wong LJ, Scaglia F, Lewis RA, Bonnen PE "Exome sequencing of a patient with suspected mitochondrial disease reveals a likely multigenic etiology." BMC Med Genet. 2013;14:83. Pubmed PMID: 23947751
- Craigen WJ "Mitochondrial DNA mutations: an overview of clinical and molecular aspects." Methods Mol Biol. 2012;837:41348. Pubmed PMID: 22215537
- Raghavan A, Sheiko T, Graham BH, Craigen WJ "Voltage-dependant anion channels: Novel insights into isoform function through genetic models." Biochim Biophys Acta. 2012 Jun;1818(6):1477-85. Pubmed PMID: 22051019
- Akman HO, Sheiko T, Tay SK, Finegold MJ, Dimauro S, Craigen WJ "Generation of a novel mouse model that recapitulates early and adult onset glycogenosis type IV." Hum Mol Genet. 2011 Nov 15;20(22):4430-9. Pubmed PMID: 21856731
- Akman HO, Raghavan A, Craigen WJ "Animal models of glycogen storage disorders." Prog Mol Biol Transl Sci. 2011;100:369-88. Pubmed PMID: 21377631
- Wong LJ, Scaglia F, Graham BH, Craigen WJ "Current molecular diagnostic algorithm for mitochondrial disorders." Mol Genet Metab. 2010 Jun;100(2):111-7. Pubmed PMID: 20359921
- Roy SS, Ehrlich AM, Craigen WJ, Hajnóczky G "VDAC2 is required for truncated BID-induced mitochondrial apoptosis by recruiting BAK to the mitochondria." EMBO Rep. 2009 Dec;10(12):1341-7. Pubmed PMID: 19820692
- Baines CP, Kaiser RA, Sheiko T, Craigen WJ, Molkentin JD "Voltage-dependent anion channels are dispensable for mitochondrial-dependent cell death." Nat Cell Biol. 2007 May;9(5):550-5. Pubmed PMID: 17417626
- Anflous-Pharayra K, Cai ZJ, Craigen WJ "VDAC1 serves as a mitochondrial binding site for hexokinase in oxidative muscles." Biochim Biophys Acta. 2007 Feb;1767(2):136-42. Pubmed PMID: 17207767
- Sabirov RZ, Sheiko T, Liu H, Deng D, Okada Y, Craigen WJ "Genetic demonstration that the plasma membrane maxianion channel and voltage-dependent anion channels are unrelated proteins." J Biol Chem. 2006 Jan 27;281(4):1897-904. Pubmed PMID: 16291750
- Cheng EH, Sheiko TV, Fisher JK, Craigen WJ, Korsmeyer SJ "VDAC2 inhibits BAK activation and mitochondrial apoptosis." Science. 2003 Jul 25;301(5632):513-7. Pubmed PMID: 12881569
- Weeber EJ, Levy M, Sampson MJ, Anflous K, Armstrong DL, Brown SE, Sweatt JD, Craigen WJ "The role of mitochondrial porins and the permeability transition pore in learning and synaptic plasticity." J Biol Chem. 2002 May 24;277(21):18891-7. Pubmed PMID: 11907043
- Anflous K, Armstrong DD, Craigen WJ "Altered mitochondrial sensitivity for ADP and maintenance of creatine-stimulated respiration in oxidative striated muscles from VDAC1-deficient mice." J Biol Chem. 2001 Jan 19;276(3):1954-60. Pubmed PMID: 11044447
Memberships
- American Society of Human Genetics
- Member
- Society for the Study of Inborn Errors of Metabolism
- Member
- American Society for the Advancement of Science
- Member
- The Biophysical Society Society for Inherited Metabolic Disorders
- Member
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