5-phosphohydroxy-L-lysine phospho-lyase; alanine-glyoxylate aminotransferase 2-like 2 (PHYKPL, AGXT2L2, PP9286)

Function

• catalyzes the pyridoxal-phosphate-dependent breakdown of 5-phosphohydroxy-L-lysine, converting it to ammonia, inorganic phosphate & 2-aminoadipate semialdehyde

(5R)-5-phosphonooxy-L-lysine + H2O = (S)-2-amino-6-oxohexanoate + NH3 + phosphate

Cofactor: pyridoxal phosphate

Kinetic parameters:

• KM=16 uM for 5-phosphohydroxy-L-lysine (at 30 degrees C)
• Vmax=256 nmol/min/mg enzyme

Structure

• homotetramer
• belongs to the class-III pyridoxal-phosphate-dependent aminotransferase family
• does not seem to possess aminotransferase activity

Compartment

mitochondria

Alternative splicing

named isoforms=3

Pathology

• mutations in PHYKPL are the cause of phosphohydroxylysinuria

More general terms

• mitochondrial protein
• ammonia lyase

Database

• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=85007
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:85007
• OMIM: https://mirror.omim.org/entry/614683
• OMIM: https://mirror.omim.org/entry/615011
• UniProt: http://www.uniprot.org/uniprot/Q8IUZ5.html