Cloning and CDNA Sequence of the Dihydrolipoamide Dehydrogenase Component Human Alpha-ketoacid Dehydrogenase Complexes

Overview

Journal Proc Natl Acad Sci U S A

Specialty Science

Date 1988 Mar 1

PMID 3278312

Citations 24

Authors

G Pons

D J Carothers

R A Pepin

A A Javed

B W Jesse

M K Ganapathi

D Samols

M S Patel

Affiliations

Soon will be listed here.

Abstract

cDNA clones comprising the entire coding region for human dihydrolipoamide dehydrogenase (dihydrolipoamide:NAD+ oxidoreductase, EC 1.8.1.4) have been isolated from a human liver cDNA library. The cDNA sequence of the largest clone consisted of 2082 base pairs and contained a 1527-base open reading frame that encodes a precursor dihydrolipoamide dehydrogenase of 509 amino acid residues. The first 35-amino acid residues of the open reading frame probably correspond to a typical mitochondrial import leader sequence. The predicted amino acid sequence of the mature protein, starting at the residue number 36 of the open reading frame, is almost identical (greater than 98% homology) with the known partial amino acid sequence of the pig heart dihydrolipoamide dehydrogenase. The cDNA clone also contains a 3' untranslated region of 505 bases with an unusual polyadenylylation signal (TATAAA) and a short poly(A) track. By blot-hybridization analysis with the cDNA as probe, two mRNAs, 2.2 and 2.4 kilobases in size, have been detected in human tissues and fibroblasts, whereas only one mRNA (2.4 kilobases) was detected in rat tissues.

Citing Articles

Dehydrogenase (DLD) Deficiency in an Iranian Patient with Recurrent Intractable Vomiting: Successful Treatment with Thiamine Supplementation.

Moosavian T, Jamalipour Soufi G, Kamfar S Iran J Child Neurol. 2024; 18(1):131-138.

PMID: 38375122 PMC: 10874511. DOI: 10.22037/ijcn.v18i1.38971.

The phenotypic spectrum of dihydrolipoamide dehydrogenase deficiency in Saudi Arabia.

Alfarsi A, Alfadhel M, Alameer S, Alhashem A, Tabarki B, Ababneh F Mol Genet Metab Rep. 2021; 29:100817.

PMID: 34745891 PMC: 8554626. DOI: 10.1016/j.ymgmr.2021.100817.

Animal models of maple syrup urine disease.

Skvorak K J Inherit Metab Dis. 2009; 32(2):229-46.

PMID: 19263237 DOI: 10.1007/s10545-009-1086-z.

Somatic mutations in the mitochondria of rheumatoid arthritis synoviocytes.

Da Sylva T, Connor A, Mburu Y, Keystone E, Wu G Arthritis Res Ther. 2005; 7(4):R844-51.

PMID: 15987486 PMC: 1175034. DOI: 10.1186/ar1752.

Maple syrup urine disease: mutation analysis in Turkish patients.

Dursun A, Henneke M, Ozgul K, Gartner J, Coskun T, Tokatli A J Inherit Metab Dis. 2002; 25(2):89-97.

PMID: 12118532 DOI: 10.1023/a:1015668425004.

References

Sakurai Y, Fekuyoshi Y, Hamada M, Hayakawa T, Koike M . Mammalian alpha-keto acid dehydrogenase complexes. VI. Nature of the multiple forms of pig heart lipoamide dehydrogenase. J Biol Chem. 1970; 245(17):4453-62. View

KIKUCHI G, Hiraga K, Yoshida T . Role of the glycine-cleavage system in glycine and serine metabolism in various organs. Biochem Soc Trans. 1980; 8(5):504-6. DOI: 10.1042/bst0080504. View

Koike M, Koike K . Structure, assembly and function of mammalian alpha-keto acid dehydrogenase complexes. Adv Biophys. 1976; :187-227. View

SANGER F, Nicklen S, Coulson A . DNA sequencing with chain-terminating inhibitors. Proc Natl Acad Sci U S A. 1977; 74(12):5463-7. PMC: 431765. DOI: 10.1073/pnas.74.12.5463. View

Pettit F, Yeaman S, Reed L . Purification and characterization of branched chain alpha-keto acid dehydrogenase complex of bovine kidney. Proc Natl Acad Sci U S A. 1978; 75(10):4881-5. PMC: 336225. DOI: 10.1073/pnas.75.10.4881. View

Kemp D, Cowman A . Direct immunoassay for detecting Escherichia coli colonies that contain polypeptides encoded by cloned DNA segments. Proc Natl Acad Sci U S A. 1981; 78(7):4520-4. PMC: 319823. DOI: 10.1073/pnas.78.7.4520. View

Vaitukaitis J . Production of antisera with small doses of immunogen: multiple intradermal injections. Methods Enzymol. 1981; 73(Pt B):46-52. DOI: 10.1016/0076-6879(81)73055-6. View

Matuda S, Saheki T . Intracellular distribution and biosynthesis of lipoamide dehydrogenase in rat liver. J Biochem. 1982; 91(2):553-61. DOI: 10.1093/oxfordjournals.jbchem.a133727. View

Packman L, Perham R . An amino acid sequence in the active site of lipoamide dehydrogenase from Bacillus stearothermophilus. FEBS Lett. 1982; 139(2):155-8. DOI: 10.1016/0014-5793(82)80839-9. View

10.

Williams Jr C, Arscott L, Schulz G . Amino acid sequence homology between pig heart lipoamide dehydrogenase and human erythrocyte glutathione reductase. Proc Natl Acad Sci U S A. 1982; 79(7):2199-201. PMC: 346158. DOI: 10.1073/pnas.79.7.2199. View

11.

Pettit F, Reed L . Pyruvate dehydrogenase complex from bovine kidney and heart. Methods Enzymol. 1982; 89 Pt D:376-86. DOI: 10.1016/s0076-6879(82)89067-8. View

12.

Messing J . New M13 vectors for cloning. Methods Enzymol. 1983; 101:20-78. DOI: 10.1016/0076-6879(83)01005-8. View

13.

Heffelfinger S, Sewell E, Danner D . Antibodies to bovine liver branched-chain 2-oxo acid dehydrogenase cross-react with this enzyme complex from other tissues and species. Biochem J. 1983; 213(2):339-44. PMC: 1152133. DOI: 10.1042/bj2130339. View

14.

Matuda S, Shirahama T, Saheki T, Miura S, Mori M . Purification and immunochemical studies of pyruvate dehydrogenase complex from rat heart, and cell-free synthesis of lipoamide dehydrogenase, a component of the complex. Biochim Biophys Acta. 1983; 741(1):86-93. DOI: 10.1016/0167-4781(83)90013-1. View

15.

Stephens P, Lewis H, Darlison M, Guest J . Nucleotide sequence of the lipoamide dehydrogenase gene of Escherichia coli K12. Eur J Biochem. 1983; 135(3):519-27. DOI: 10.1111/j.1432-1033.1983.tb07683.x. View

16.

Rice D, Schulz G, Guest J . Structural relationship between glutathione reductase and lipoamide dehydrogenase. J Mol Biol. 1984; 174(3):483-96. DOI: 10.1016/0022-2836(84)90332-2. View

17.

Hod Y, Morris S, Hanson R . Induction by cAMP of the mRNA encoding the cytosolic form of phosphoenolpyruvate carboxykinase (GTP) from the chicken. Identification and characterization of a cDNA clone for the enzyme. J Biol Chem. 1984; 259(24):15603-8. View

18.

Cook K, Bradford A, Yeaman S . Resolution and reconstitution of bovine kidney branched-chain 2-oxo acid dehydrogenase complex. Biochem J. 1985; 225(3):731-5. PMC: 1144650. DOI: 10.1042/bj2250731. View

19.

Birnstiel M, Busslinger M, Strub K . Transcription termination and 3' processing: the end is in site!. Cell. 1985; 41(2):349-59. DOI: 10.1016/s0092-8674(85)80007-6. View

20.

De Marcucci O, Hunter A, Lindsay J . Low immunogenicity of the common lipoamide dehydrogenase subunit (E3) of mammalian pyruvate dehydrogenase and 2-oxoglutarate dehydrogenase multienzyme complexes. Biochem J. 1985; 226(2):509-17. PMC: 1144738. DOI: 10.1042/bj2260509. View