PMID- 6777600
OWN - NLM
STAT- MEDLINE
DCOM- 19810226
LR  - 20190829
IS  - 0141-8955 (Print)
IS  - 0141-8955 (Linking)
VI  - 3
IP  - 2
DP  - 1980
TI  - Argininosuccinic acid synthetase deficiency in a hamster cell line and its 
      complementation of argininosuccinic aciduria human fibroblasts.
PG  - 45-8
AB  - Unlike normal human cells, cultured fibroblasts from patients with 
      argininosuccinic aciduria cannot synthesize arginine from citrulline because they 
      have a deficiency of argininosuccinic acid lyase (ASL). We have found that V79, a 
      Chinese hamster cell line, cannot grow on citrulline. Although these cells show a 
      normal uptake of citrulline and have levels of ASL comparable to a human cell 
      line (HeLa) which can grow in citrulline-containing medium, V79 cells have less 
      than 5% of the argininosuccinic acid synthetase (ASS) activity of HeLa and cannot 
      convert citrulline to argininosuccinate and thence to arginine. When 
      heterokaryocytes are formed between V79 and a human cell line derived from a 
      patient with ASL deficiency, complementation takes place and citrulline is 
      incorporated into cell protein, presumably after having been converted to 
      arginine. This is the first time that a genetic defect of the urea cycle has been 
      corrected in human cells.
FAU - Gonzalez-Noriega, A
AU  - Gonzalez-Noriega A
FAU - Verduzco, J
AU  - Verduzco J
FAU - Prieto, E
AU  - Prieto E
FAU - Velazquez, A
AU  - Velazquez A
LA  - eng
PT  - Journal Article
PL  - United States
TA  - J Inherit Metab Dis
JT  - Journal of inherited metabolic disease
JID - 7910918
RN  - 2387-71-5 (Argininosuccinic Acid)
RN  - 29VT07BGDA (Citrulline)
RN  - 94ZLA3W45F (Arginine)
RN  - EC 4.- (Lyases)
RN  - EC 4.3.2.1 (Argininosuccinate Lyase)
RN  - EC 6.- (Ligases)
RN  - EC 6.3.4.5 (Argininosuccinate Synthase)
SB  - IM
MH  - Animals
MH  - Arginine/*analogs & derivatives
MH  - Argininosuccinate Lyase/genetics/*metabolism
MH  - Argininosuccinate Synthase/*deficiency/genetics
MH  - Argininosuccinic Acid/*urine
MH  - Cell Line
MH  - Citrulline/metabolism
MH  - Cricetinae
MH  - Female
MH  - Fibroblasts/enzymology
MH  - Genes, Recessive
MH  - Genetic Complementation Test
MH  - HeLa Cells
MH  - Humans
MH  - Hybrid Cells
MH  - Ligases/*deficiency
MH  - Liver/enzymology
MH  - Lyases/*metabolism
MH  - Male
MH  - Renal Aminoacidurias/*metabolism
EDAT- 1980/01/01 00:00
MHDA- 1980/01/01 00:01
CRDT- 1980/01/01 00:00
PHST- 1980/01/01 00:00 [pubmed]
PHST- 1980/01/01 00:01 [medline]
PHST- 1980/01/01 00:00 [entrez]
AID - 10.1007/BF02312521 [doi]
PST - ppublish
SO  - J Inherit Metab Dis. 1980;3(2):45-8. doi: 10.1007/BF02312521.