Name5-phosphohydroxy-L-lysine phospho-lyase
Synonyms
  • 4.2.3.134
  • AGXT2L2
  • Alanine--glyoxylate aminotransferase 2-like 2
Gene NamePHYKPL
OrganismHuman
Amino acid sequence
>lcl|BSEQ0004246|5-phosphohydroxy-L-lysine phospho-lyase
MAADQRPKADTLALRQRLISSSCRLFFPEDPVKIVRAQGQYMYDEQGAEYIDCISNVAHV
GHCHPLVVQAAHEQNQVLNTNSRYLHDNIVDYAQRLSETLPEQLCVFYFLNSGSEANDLA
LRLARHYTGHQDVVVLDHAYHGHLSSLIDISPYKFRNLDGQKEWVHVAPLPDTYRGPYRE
DHPNPAMAYANEVKRVVSSAQEKGRKIAAFFAESLPSVGGQIIPPAGYFSQVAEHIRKAG
GVFVADEIQVGFGRVGKHFWAFQLQGKDFVPDIVTMGKSIGNGHPVACVAATQPVARAFE
ATGVEYFNTFGGSPVSCAVGLAVLNVLEKEQLQDHATSVGSFLMQLLGQQKIKHPIVGDV
RGVGLFIGVDLIKDEATRTPATEEAAYLVSRLKENYVLLSTDGPGRNILKFKPPMCFSLD
NARQVVAKLDAILTDMEEKVRSCETLRLQP
Number of residues450
Molecular Weight49710.245
Theoretical pI6.76
GO Classification
Functions
  • transaminase activity
  • identical protein binding
  • lyase activity
  • pyridoxal phosphate binding
Processes
  • cellular nitrogen compound metabolic process
  • small molecule metabolic process
  • collagen catabolic process
  • extracellular matrix disassembly
  • extracellular matrix organization
  • lysine catabolic process
Components
  • mitochondrial matrix
General FunctionTransaminase activity
Specific FunctionCatalyzes the pyridoxal-phosphate-dependent breakdown of 5-phosphohydroxy-L-lysine, converting it to ammonia, inorganic phosphate and 2-aminoadipate semialdehyde.
Pfam Domain Function
Transmembrane RegionsNot Available
GenBank Protein ID18027842
UniProtKB IDQ8IUZ5
UniProtKB Entry NameAT2L2_HUMAN
Cellular LocationMitochondrion
Gene sequence
>lcl|BSEQ0020593|5-phosphohydroxy-L-lysine phospho-lyase (PHYKPL)
ATGTACGATGAACAGGGGGCAGAATACATCGATTGCATCAGCAATGTGGCGCACGTTGGG
CACTGCCACCCTCTCGTGGTCCAAGCAGCACATGAGCAGAACCAGGTGCTCAACACCAAC
AGCCGGTACCTGCATGACAACATCGTGGACTATGCGCAGAGGCTGTCAGAGACCCTGCCG
GAGCAGCTCTGTGTGTTCTATTTCCTGAATTCTGGGTCAGAAGCCAATGACCTGGCCCTG
AGGCTGGCTCGCCACTACACGGGACACCAGGACGTGGTGGTATTAGATCATGCGTATCAC
GGCCACCTGAGCTCCCTGATTGACATCAGTCCCTACAAGTTCCGCAACCTGGATGGCCAG
AAGGAGTGGGTCCACGTGGCACCTCTCCCAGACACCTACCGGGGCCCCTACCGGGAGGAC
CACCCCAACCCAGCTATGGCCTATGCCAACGAGGTGAAACGTGTGGTCAGCAGTGCACAG
GAGAAGGGCAGGAAGATTGCAGCCTTCTTCGCTGAGTCTCTGCCCAGTGTGGGAGGGCAG
ATCATTCCCCCTGCTGGCTACTTCTCCCAAGTGGCAGAGCACATCCGCAAGGCCGGAGGG
GTCTTTGTTGCAGATGAGATCCAGGTTGGCTTTGGCCGGGTAGGCAAGCACTTCTGGGCC
TTCCAGCTCCAGGGAAAAGACTTCGTCCCTGACATCGTCACCATGGGCAAGTCCATTGGC
AACGGCCACCCTGTTGCCTGCGTGGCCGCAACCCAGCCTGTGGCGAGGGCATTTGAAGCC
ACCGGCGTTGAGTACTTCAACACGTTTGGGGGCAGCCCAGTGTCCTGCGCTGTGGGGCTG
GCCGTCCTGAATGTCTTGGAGAAGGAGCAGCTCCAGGATCATGCCACCAGTGTAGGCAGC
TTCCTGATGCAGCTCCTCGGGCAGCAAAAAATCAAACATCCCATCGTCGGGGATGTCAGG
GGTGTTGGGCTCTTCATTGGTGTGGATCTGATCAAAGATGAGGCCACAAGGACACCAGCA
ACTGAAGAGGCTGCCTACTTGGTATCAAGGCTGAAGGAGAACTACGTTTTGCTGAGCACT
GATGGCCCTGGGAGGAACATCCTGAAGTTTAAGCCCCCAATGTGCTTCAGCCTGGACAAT
GCACGGCAGGTGGTGGCAAAGCTGGATGCCATTCTGACTGACATGGAAGAGAAGGTGAGA
AGTTGTGAAACGCTGAGGCTCCAGCCCTAA
GenBank Gene IDAF318375
GeneCard IDNot Available
GenAtlas IDAGXT2L2
HGNC IDHGNC:28249
Chromosome Location5
Locus5q35.3
References
  1. Wan D, Gong Y, Qin W, Zhang P, Li J, Wei L, Zhou X, Li H, Qiu X, Zhong F, He L, Yu J, Yao G, Jiang H, Qian L, Yu Y, Shu H, Chen X, Xu H, Guo M, Pan Z, Chen Y, Ge C, Yang S, Gu J: Large-scale cDNA transfection screening for genes related to cancer development and progression. Proc Natl Acad Sci U S A. 2004 Nov 2;101(44):15724-9. Epub 2004 Oct 21. 15498874
  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. 14702039
  3. Schmutz J, Martin J, Terry A, Couronne O, Grimwood J, Lowry S, Gordon LA, Scott D, Xie G, Huang W, Hellsten U, Tran-Gyamfi M, She X, Prabhakar S, Aerts A, Altherr M, Bajorek E, Black S, Branscomb E, Caoile C, Challacombe JF, Chan YM, Denys M, Detter JC, Escobar J, Flowers D, Fotopulos D, Glavina T, Gomez M, Gonzales E, Goodstein D, Grigoriev I, Groza M, Hammon N, Hawkins T, Haydu L, Israni S, Jett J, Kadner K, Kimball H, Kobayashi A, Lopez F, Lou Y, Martinez D, Medina C, Morgan J, Nandkeshwar R, Noonan JP, Pitluck S, Pollard M, Predki P, Priest J, Ramirez L, Retterer J, Rodriguez A, Rogers S, Salamov A, Salazar A, Thayer N, Tice H, Tsai M, Ustaszewska A, Vo N, Wheeler J, Wu K, Yang J, Dickson M, Cheng JF, Eichler EE, Olsen A, Pennacchio LA, Rokhsar DS, Richardson P, Lucas SM, Myers RM, Rubin EM: The DNA sequence and comparative analysis of human chromosome 5. Nature. 2004 Sep 16;431(7006):268-74. 15372022
  4. 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. 15489334
  5. Veiga-da-Cunha M, Hadi F, Balligand T, Stroobant V, Van Schaftingen E: Molecular identification of hydroxylysine kinase and of ammoniophospholyases acting on 5-phosphohydroxy-L-lysine and phosphoethanolamine. J Biol Chem. 2012 Mar 2;287(10):7246-55. doi: 10.1074/jbc.M111.323485. Epub 2012 Jan 12. 22241472
  6. Bian Y, Song C, Cheng K, Dong M, Wang F, Huang J, Sun D, Wang L, Ye M, Zou H: An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome. J Proteomics. 2014 Jan 16;96:253-62. doi: 10.1016/j.jprot.2013.11.014. Epub 2013 Nov 22. 24275569
  7. Veiga-da-Cunha M, Verhoeven-Duif NM, de Koning TJ, Duran M, Dorland B, Van Schaftingen E: Mutations in the AGXT2L2 gene cause phosphohydroxylysinuria. J Inherit Metab Dis. 2013 Nov;36(6):961-6. doi: 10.1007/s10545-012-9568-9. Epub 2012 Dec 14. 23242558