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Literature summary for 2.6.1.44 extracted from

  • Cellini, B.; Montioli, R.; Voltattorni, C.B.
    Human liver peroxisomal alanine:glyoxylate aminotransferase: characterization of the two allelic forms and their pathogenic variants (2011), Biochim. Biophys. Acta, 1814, 1577-1584.
    View publication on PubMed

Cloned(Commentary)

Cloned (Comment) Organism
expressed in Escherichia coli Homo sapiens

Crystallization (Commentary)

Crystallization (Comment) Organism
-
Homo sapiens

Protein Variants

Protein Variants Comment Organism
F152I soluble, catalytically active Homo sapiens
G41R naturally occuring mutation, predicted to be responsible for the depletion of immunoreactive enzyme protein and formation of intraperoxisomal aggregates Homo sapiens
G41V naturally occuring mutation Homo sapiens
G82E naturally occuring mutation, decreased catalytic activity Homo sapiens
P11L/I340M minor allele, naturally occuring variant; mutant protein (minor allel) is about 95% peroxisomal and 5% mitochondrial; P11/I340 major allele is 100% peroxisomal, minor allele in both the holo and apo forms is more sensitive to thermal denaturation and to urea unfolding than major allele Homo sapiens
P11L/I340M/F152I naturally occuring mutation, possibly mistargeting into mitochondrial matrix Homo sapiens
P11L/I340M/G41R naturally occuring mutation, predicted to be responsible for the depletion of immunoreactive enzyme protein and formation of intraperoxisomal aggregates Homo sapiens

KM Value [mM]

KM Value [mM] KM Value Maximum [mM] Substrate Comment Organism Structure
0.13
-
glyoxylate G41V, pH not specified in the publication, temperature not specified in the publication Homo sapiens
0.15
-
glyoxylate G82E, pH not specified in the publication, temperature not specified in the publication Homo sapiens
0.22
-
glyoxylate P11L/I340M minor allele, pH not specified in the publication, temperature not specified in the publication Homo sapiens
0.23
-
glyoxylate major allele (P11, I340), pH not specified in the publication, temperature not specified in the publication Homo sapiens
0.25
-
glyoxylate P11L/I340M/F152I, pH not specified in the publication, temperature not specified in the publication Homo sapiens
0.28
-
glyoxylate F152I, pH not specified in the publication, temperature not specified in the publication Homo sapiens
0.32
-
glyoxylate P11L/I340M/G41R, pH not specified in the publication, temperature not specified in the publication Homo sapiens
0.41
-
glyoxylate G41R, pH not specified in the publication, temperature not specified in the publication Homo sapiens
15
-
L-alanine G82E, pH not specified in the publication, temperature not specified in the publication Homo sapiens
22
-
L-alanine G41R, pH not specified in the publication, temperature not specified in the publication Homo sapiens
28
-
L-alanine P11L/I340M minor allele, pH not specified in the publication, temperature not specified in the publication Homo sapiens
30
-
L-alanine P11L/I340M/G41R, pH not specified in the publication, temperature not specified in the publication Homo sapiens
31
-
L-alanine major allele (P11, I340), pH not specified in the publication, temperature not specified in the publication Homo sapiens
37
-
L-alanine F152I, pH not specified in the publication, temperature not specified in the publication Homo sapiens
41
-
L-alanine P11L/I340M/F152I, pH not specified in the publication, temperature not specified in the publication Homo sapiens
42
-
L-alanine G41V, pH not specified in the publication, temperature not specified in the publication Homo sapiens

Localization

Localization Comment Organism GeneOntology No. Textmining
peroxisome
-
Homo sapiens 5777
-

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
-
-

Purification (Commentary)

Purification (Comment) Organism
-
Homo sapiens

Source Tissue

Source Tissue Comment Organism Textmining
liver
-
Homo sapiens
-

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
L-alanine + glyoxylate
-
Homo sapiens pyruvate + glycine
-
r

Subunits

Subunits Comment Organism
homodimer
-
Homo sapiens

Synonyms

Synonyms Comment Organism
alanine:glyoxylate aminotransferase
-
Homo sapiens

Turnover Number [1/s]

Turnover Number Minimum [1/s] Turnover Number Maximum [1/s] Substrate Comment Organism Structure
0.068
-
glyoxylate G82E, pH not specified in the publication, temperature not specified in the publication Homo sapiens
0.07
-
L-alanine G82E, pH not specified in the publication, temperature not specified in the publication Homo sapiens
10.6
-
L-alanine P11L/I340M/G41R, pH not specified in the publication, temperature not specified in the publication Homo sapiens
11.1
-
glyoxylate P11L/I340M/G41R, pH not specified in the publication, temperature not specified in the publication Homo sapiens
17.1
-
L-alanine G41V, pH not specified in the publication, temperature not specified in the publication Homo sapiens
18.3
-
glyoxylate G41V, pH not specified in the publication, temperature not specified in the publication Homo sapiens
19.8
-
L-alanine G41R, pH not specified in the publication, temperature not specified in the publication Homo sapiens
20.5
-
glyoxylate G41R, pH not specified in the publication, temperature not specified in the publication Homo sapiens
33
-
L-alanine P11L/I340M minor allele, pH not specified in the publication, temperature not specified in the publication Homo sapiens
33.6
-
L-alanine P11L/I340M/F152I, pH not specified in the publication, temperature not specified in the publication Homo sapiens
35
-
L-alanine F152I, pH not specified in the publication, temperature not specified in the publication Homo sapiens
37
-
glyoxylate P11L/I340M minor allele, pH not specified in the publication, temperature not specified in the publication Homo sapiens
39
-
glyoxylate F152I, pH not specified in the publication, temperature not specified in the publication Homo sapiens
40
-
glyoxylate P11L/I340M/F152I, pH not specified in the publication, temperature not specified in the publication Homo sapiens
45
-
glyoxylate major allele (P11, I340), pH not specified in the publication, temperature not specified in the publication Homo sapiens
45
-
L-alanine major allele (P11, I340), pH not specified in the publication, temperature not specified in the publication Homo sapiens

Cofactor

Cofactor Comment Organism Structure
pyridoxal 5'-phosphate 2 mol per dimer Homo sapiens

General Information

General Information Comment Organism
malfunction deficiency is responsible for Primary Hyperoxaluria Type 1, an autosomal recessive disorder Homo sapiens

kcat/KM [mM/s]

kcat/KM Value [1/mMs-1] kcat/KM Value Maximum [1/mMs-1] Substrate Comment Organism Structure
0.005
-
L-alanine G82E, pH not specified in the publication, temperature not specified in the publication Homo sapiens
0.35
-
L-alanine P11L/I340M/G41R, pH not specified in the publication, temperature not specified in the publication Homo sapiens
0.41
-
L-alanine G41V, pH not specified in the publication, temperature not specified in the publication Homo sapiens
0.45
-
glyoxylate G82E, pH not specified in the publication, temperature not specified in the publication Homo sapiens
0.82
-
L-alanine P11L/I340M/F152I, pH not specified in the publication, temperature not specified in the publication Homo sapiens
0.9
-
L-alanine G41R, pH not specified in the publication, temperature not specified in the publication Homo sapiens
0.95
-
L-alanine F152I, pH not specified in the publication, temperature not specified in the publication Homo sapiens
1.2
-
L-alanine P11L/I340M minor allele, pH not specified in the publication, temperature not specified in the publication Homo sapiens
1.4
-
L-alanine major allele (P11, I340), pH not specified in the publication, temperature not specified in the publication Homo sapiens
35
-
glyoxylate P11L/I340M/G41R, pH not specified in the publication, temperature not specified in the publication Homo sapiens
50
-
glyoxylate G41R, pH not specified in the publication, temperature not specified in the publication Homo sapiens
139
-
glyoxylate F152I, pH not specified in the publication, temperature not specified in the publication Homo sapiens
143
-
glyoxylate G41V, pH not specified in the publication, temperature not specified in the publication Homo sapiens
160
-
glyoxylate P11L/I340M/F152I, pH not specified in the publication, temperature not specified in the publication Homo sapiens
168
-
glyoxylate P11L/I340M minor allele, pH not specified in the publication, temperature not specified in the publication Homo sapiens
196
-
glyoxylate major allele (P11, I340), pH not specified in the publication, temperature not specified in the publication Homo sapiens