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Identification
HMDB Protein ID HMDBP04991
Secondary Accession Numbers
  • 10586
Name 3-hydroxymethyl-3-methylglutaryl-CoA lyase, cytoplasmic
Synonyms
  1. 3-hydroxymethyl-3-methylglutaryl-CoA lyase-like protein 1
  2. Endoplasmic reticulum 3-hydroxymethyl-3-methylglutaryl-CoA lyase
  3. er-cHL
Gene Name HMGCLL1
Protein Type Enzyme
Biological Properties
General Function Involved in catalytic activity
Specific Function Non-mitochondrial 3-hydroxymethyl-3-methylglutaryl-CoA lyase that catalyzes a cation-dependent cleavage of (S)-3-hydroxy-3-methylglutaryl-CoA into acetyl-CoA and acetoacetate, a key step in ketogenesis, the products of which support energy production in nonhepatic animal tissues.
Pathways
  • (S)-3-hydroxy-3-methylglutaryl-CoA degradation
Reactions
3-Hydroxy-3-methylglutaryl-CoA → Acetyl-CoA + Acetoacetic acid details
GO Classification
Biological Process
ketone body biosynthetic process
Cellular Component
endoplasmic reticulum membrane
cytosol
endoplasmic reticulum
perinuclear region of cytoplasm
membrane
Function
catalytic activity
Molecular Function
metal ion binding
hydroxymethylglutaryl-CoA lyase activity
Process
metabolic process
Cellular Location Not Available
Gene Properties
Chromosome Location 6
Locus 6p12.1
SNPs HMGCLL1
Gene Sequence
>1113 bp
ATGGGGAATGTGCCATCCGCGGTGAAGCACTGCCTCAGCTACCAGCAGCTTCTCCGGGAG
CATCTCTGGATCGGGGATTCAGTGGCAGGGGCGCTCGACCCCGCGCAGACTTCTCTTCTA
ACAAACCTTCACTGCTTTCAGCCCGATGTCTCTGGCTTCTCAGTCTCCTTGGCAGGCACG
GTGGCTTGTATCCACTGGGAAACATCCCAGTTATCTGGACTCCCTGAGTTTGTTAAAATA
GTAGAAGTTGGGCCTAGGGATGGATTGCAGAATGAAAAGGTTATAGTTCCTACAGATATA
AAAATTGAATTTATCAATCGACTTTCCCAAACTGGCTTGTCTGTAATAGAAGTGACTAGC
TTTGTGTCTTCCAGATGGGTACCACAGATGGCTGATCACACTGAAGTAATGAAAGGCATT
CATCAATATCCAGGAGTTCGCTATCCTGTCCTTACTCCTAATCTTCAGGGTTTTCACCAT
GCTGTTGCTGCTGGAGCTACTGAGATATCAGTTTTTGGAGCTGCATCTGAATCCTTTAGC
AAGAAGAATATTAACTGTTCCATTGAAGAAAGTATGGGAAAATTTGAGGAGGTTGTTAAG
TCTGCAAGACACATGAATATTCCAGCACGAGGGTATGTGTCTTGTGCTCTGGGCTGTCCA
TATGAAGGAAGTATTACACCGCAAAAAGTGACAGAAGTGTCTAAGAGATTGTACGGCATG
GGTTGTTATGAGATCTCTCTAGGAGACACAATTGGAGTGGGAACTCCAGGAAGTATGAAA
AGAATGTTGGAAAGTGTGATGAAAGAAATCCCACCAGGTGCTCTTGCTGTTCACTGTCAT
GACACATACGGACAAGCCTTAGCAAATATCCTTACGGCCCTTCAGATGGGAATTAATGTG
GTGGACTCCGCAGTATCCGGATTAGGTGGCTGCCCTTATGCAAAAGGTGCTTCTGGGAAT
GTAGCCACTGAGGATTTGATATATATGCTTAATGGCCTGGGGCTCAATACAGGTGTGAAT
CTATACAAAGTGATGGAAGCTGGTGACTTTATTTGCAAAGCTGTGAATAAAACCACAAAC
TCTAAAGTAGCACAAGCCTCCTTCAATGCTTGA
Protein Properties
Number of Residues 370
Molecular Weight 36327.465
Theoretical pI 6.605
Pfam Domain Function
Signals Not Available
Transmembrane Regions Not Available
Protein Sequence
>Probable 3-hydroxymethyl-3-methylglutaryl-CoA lyase 2
MGNVPSAVKHCLSYQQLLREHLWIGDSVAGALDPAQTSLLTNLHCFQPDVSGFSVSLAGT
VACIHWETSQLSGLPEFVKIVEVGPRDGLQNEKVIVPTDIKIEFINRLSQTGLSVIEVTS
FVSSRWVPQMADHTEVMKGIHQYPGVRYPVLTPNLQGFHHAVAAGATEISVFGAASESFS
KKNINCSIEESMGKFEEVVKSARHMNIPARGYVSCALGCPYEGSITPQKVTEVSKRLYGM
GCYEISLGDTIGVGTPGSMKRMLESVMKEIPPGALAVHCHDTYGQALANILTALQMGINV
VDSAVSGLGGCPYAKGASGNVATEDLIYMLNGLGLNTGVNLYKVMEAGDFICKAVNKTTN
SKVAQASFNA
GenBank ID Protein 109150427
UniProtKB/Swiss-Prot ID Q8TB92
UniProtKB/Swiss-Prot Entry Name HMGC2_HUMAN
PDB IDs Not Available
GenBank Gene ID NM_019036.2
GeneCard ID HMGCLL1
GenAtlas ID HMGCLL1
HGNC ID HGNC:21359
References
General References
  1. Mungall AJ, Palmer SA, Sims SK, Edwards CA, Ashurst JL, Wilming L, Jones MC, Horton R, Hunt SE, Scott CE, Gilbert JG, Clamp ME, Bethel G, Milne S, Ainscough R, Almeida JP, Ambrose KD, Andrews TD, Ashwell RI, Babbage AK, Bagguley CL, Bailey J, Banerjee R, Barker DJ, Barlow KF, Bates K, Beare DM, Beasley H, Beasley O, Bird CP, Blakey S, Bray-Allen S, Brook J, Brown AJ, Brown JY, Burford DC, Burrill W, Burton J, Carder C, Carter NP, Chapman JC, Clark SY, Clark G, Clee CM, Clegg S, Cobley V, Collier RE, Collins JE, Colman LK, Corby NR, Coville GJ, Culley KM, Dhami P, Davies J, Dunn M, Earthrowl ME, Ellington AE, Evans KA, Faulkner L, Francis MD, Frankish A, Frankland J, French L, Garner P, Garnett J, Ghori MJ, Gilby LM, Gillson CJ, Glithero RJ, Grafham DV, Grant M, Gribble S, Griffiths C, Griffiths M, Hall R, Halls KS, Hammond S, Harley JL, Hart EA, Heath PD, Heathcott R, Holmes SJ, Howden PJ, Howe KL, Howell GR, Huckle E, Humphray SJ, Humphries MD, Hunt AR, Johnson CM, Joy AA, Kay M, Keenan SJ, Kimberley AM, King A, Laird GK, Langford C, Lawlor S, Leongamornlert DA, Leversha M, Lloyd CR, Lloyd DM, Loveland JE, Lovell J, Martin S, Mashreghi-Mohammadi M, Maslen GL, Matthews L, McCann OT, McLaren SJ, McLay K, McMurray A, Moore MJ, Mullikin JC, Niblett D, Nickerson T, Novik KL, Oliver K, Overton-Larty EK, Parker A, Patel R, Pearce AV, Peck AI, Phillimore B, Phillips S, Plumb RW, Porter KM, Ramsey Y, Ranby SA, Rice CM, Ross MT, Searle SM, Sehra HK, Sheridan E, Skuce CD, Smith S, Smith M, Spraggon L, Squares SL, Steward CA, Sycamore N, Tamlyn-Hall G, Tester J, Theaker AJ, Thomas DW, Thorpe A, Tracey A, Tromans A, Tubby B, Wall M, Wallis JM, West AP, White SS, Whitehead SL, Whittaker H, Wild A, Willey DJ, Wilmer TE, Wood JM, Wray PW, Wyatt JC, Young L, Younger RM, Bentley DR, Coulson A, Durbin R, Hubbard T, Sulston JE, Dunham I, Rogers J, Beck S: The DNA sequence and analysis of human chromosome 6. Nature. 2003 Oct 23;425(6960):805-11. [PubMed:14574404 ]
  2. Ota T, Suzuki Y, Nishikawa T, Otsuki T, Sugiyama T, Irie R, Wakamatsu A, Hayashi K, Sato H, Nagai K, Kimura K, Makita H, Sekine M, Obayashi M, Nishi T, Shibahara T, Tanaka T, Ishii S, Yamamoto J, Saito K, Kawai Y, Isono Y, Nakamura Y, Nagahari K, Murakami K, Yasuda T, Iwayanagi T, Wagatsuma M, Shiratori A, Sudo H, Hosoiri T, Kaku Y, Kodaira H, Kondo H, Sugawara M, Takahashi M, Kanda K, Yokoi T, Furuya T, Kikkawa E, Omura Y, Abe K, Kamihara K, Katsuta N, Sato K, Tanikawa M, Yamazaki M, Ninomiya K, Ishibashi T, Yamashita H, Murakawa K, Fujimori K, Tanai H, Kimata M, Watanabe M, Hiraoka S, Chiba Y, Ishida S, Ono Y, Takiguchi S, Watanabe S, Yosida M, Hotuta T, Kusano J, Kanehori K, Takahashi-Fujii A, Hara H, Tanase TO, Nomura Y, Togiya S, Komai F, Hara R, Takeuchi K, Arita M, Imose N, Musashino K, Yuuki H, Oshima A, Sasaki N, Aotsuka S, Yoshikawa Y, Matsunawa H, Ichihara T, Shiohata N, Sano S, Moriya S, Momiyama H, Satoh N, Takami S, Terashima Y, Suzuki O, Nakagawa S, Senoh A, Mizoguchi H, Goto Y, Shimizu F, Wakebe H, Hishigaki H, Watanabe T, Sugiyama A, Takemoto M, Kawakami B, Yamazaki M, Watanabe K, Kumagai A, Itakura S, Fukuzumi Y, Fujimori Y, Komiyama M, Tashiro H, Tanigami A, Fujiwara T, Ono T, Yamada K, Fujii Y, Ozaki K, Hirao M, Ohmori Y, Kawabata A, Hikiji T, Kobatake N, Inagaki H, Ikema Y, Okamoto S, Okitani R, Kawakami T, Noguchi S, Itoh T, Shigeta K, Senba T, Matsumura K, Nakajima Y, Mizuno T, Morinaga M, Sasaki M, Togashi T, Oyama M, Hata H, Watanabe M, Komatsu T, Mizushima-Sugano J, Satoh T, Shirai Y, Takahashi Y, Nakagawa K, Okumura K, Nagase T, Nomura N, Kikuchi H, Masuho Y, Yamashita R, Nakai K, Yada T, Nakamura Y, Ohara O, Isogai T, Sugano S: Complete sequencing and characterization of 21,243 full-length human cDNAs. Nat Genet. 2004 Jan;36(1):40-5. Epub 2003 Dec 21. [PubMed:14702039 ]
  3. Gerhard DS, Wagner L, Feingold EA, Shenmen CM, Grouse LH, Schuler G, Klein SL, Old S, Rasooly R, Good P, Guyer M, Peck AM, Derge JG, Lipman D, Collins FS, Jang W, Sherry S, Feolo M, Misquitta L, Lee E, Rotmistrovsky K, Greenhut SF, Schaefer CF, Buetow K, Bonner TI, Haussler D, Kent J, Kiekhaus M, Furey T, Brent M, Prange C, Schreiber K, Shapiro N, Bhat NK, Hopkins RF, Hsie F, Driscoll T, Soares MB, Casavant TL, Scheetz TE, Brown-stein MJ, Usdin TB, Toshiyuki S, Carninci P, Piao Y, Dudekula DB, Ko MS, Kawakami K, Suzuki Y, Sugano S, Gruber CE, Smith MR, Simmons B, Moore T, Waterman R, Johnson SL, Ruan Y, Wei CL, Mathavan S, Gunaratne PH, Wu J, Garcia AM, Hulyk SW, Fuh E, Yuan Y, Sneed A, Kowis C, Hodgson A, Muzny DM, McPherson J, Gibbs RA, Fahey J, Helton E, Ketteman M, Madan A, Rodrigues S, Sanchez A, Whiting M, Madari A, Young AC, Wetherby KD, Granite SJ, Kwong PN, Brinkley CP, Pearson RL, Bouffard GG, Blakesly RW, Green ED, Dickson MC, Rodriguez AC, Grimwood J, Schmutz J, Myers RM, Butterfield YS, Griffith M, Griffith OL, Krzywinski MI, Liao N, Morin R, Palmquist D, Petrescu AS, Skalska U, Smailus DE, Stott JM, Schnerch A, Schein JE, Jones SJ, Holt RA, Baross A, Marra MA, Clifton S, Makowski KA, Bosak S, Malek J: The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC). Genome Res. 2004 Oct;14(10B):2121-7. [PubMed:15489334 ]