EC Number |
Recommended Name |
Source Tissue |
Reference |
---|
6.1.1.16 | cysteine-tRNA ligase |
fibroblast |
cultured |
194 |
6.1.1.16 | cysteine-tRNA ligase |
fibroblast |
from patients with cystinosis |
194 |
6.2.1.15 | arachidonate-CoA ligase |
fibroblast |
- |
640 |
6.2.1.16 | acetoacetate-CoA ligase |
fibroblast |
- |
650 |
6.2.1.24 | phytanate-CoA ligase |
fibroblast |
skin, cell culture |
705 |
6.3.4.10 | biotin-[propionyl-CoA-carboxylase (ATP-hydrolysing)] ligase |
fibroblast |
- |
1392, 705293 |
6.3.4.15 | biotin-[biotin carboxyl-carrier protein] ligase |
fibroblast |
cultured |
1446 |
6.3.4.7 | ribose-5-phosphate-ammonia ligase |
fibroblast |
wild-type and mutant cell line deficient in phosphoribosyldiphosphate amidotransferase and phosphoribosyldiphosphate aminotransferase activities with normal activity of ribose-5-phosphate aminotransferase activities |
1617 |
6.5.1.1 | DNA ligase (ATP) |
fibroblast |
- |
1928, 653386, 694437, 709934 |
5.1.3.14 | UDP-N-acetylglucosamine 2-epimerase (non-hydrolysing) |
fibroblast |
skin |
2275 |
5.1.3.19 | chondroitin-glucuronate 5-epimerase |
fibroblast |
skin |
2291, 2299, 2300, 2302, 2303, 2304 |
5.1.3.19 | chondroitin-glucuronate 5-epimerase |
fibroblast |
embryonic |
2299, 2304 |
5.1.3.19 | chondroitin-glucuronate 5-epimerase |
fibroblast |
30-50% decreased activity in fibroblasts treated with high xyloside concentrations |
2303 |
5.1.99.4 | alpha-methylacyl-CoA racemase |
fibroblast |
- |
2447 |
5.3.3.2 | isopentenyl-diphosphate DELTA-isomerase |
fibroblast |
in normal fibroblasts and Zellweger fibroblasts, in which the synthesis of cholesterol is impaired |
2959 |
5.3.4.1 | protein disulfide-isomerase |
fibroblast |
lung |
3076, 439186 |
5.3.99.3 | prostaglandin-E synthase |
fibroblast |
pEJ-ras transformed |
3132 |
5.3.99.5 | thromboxane-A synthase |
fibroblast |
low activity |
3183 |
4.1.2.27 | sphinganine-1-phosphate aldolase |
fibroblast |
- |
5119, 747522 |
4.1.2.27 | sphinganine-1-phosphate aldolase |
fibroblast |
skin |
5120 |
4.1.2.4 | deoxyribose-phosphate aldolase |
fibroblast |
- |
5181 |
4.1.3.4 | hydroxymethylglutaryl-CoA lyase |
fibroblast |
skin, cultured |
5374 |
3.4.21.68 | t-Plasminogen activator |
fibroblast |
embryonic lung diploid cells, IMR-90 cells, ATCC, CCL186 |
29594 |
4.2.1.18 | methylglutaconyl-CoA hydratase |
fibroblast |
- |
33741 |
4.6.1.1 | adenylate cyclase |
fibroblast |
embryo |
34776 |
4.6.1.1 | adenylate cyclase |
fibroblast |
normal and Rous sarcoma transformed |
34776 |
4.98.1.1 | protoporphyrin ferrochelatase |
fibroblast |
cultured |
34970 |
3.4.14.5 | dipeptidyl-peptidase IV |
fibroblast |
normal, 3T3, and transformed, 3T12 |
36209 |
3.4.14.5 | dipeptidyl-peptidase IV |
fibroblast |
- |
36225, 695876, 703784 |
3.4.16.2 | lysosomal Pro-Xaa carboxypeptidase |
fibroblast |
- |
36268 |
3.4.16.2 | lysosomal Pro-Xaa carboxypeptidase |
fibroblast |
foreskin fibroblast |
36269 |
3.4.17.3 | lysine carboxypeptidase |
fibroblast |
foreskin |
36393 |
3.4.23.5 | cathepsin D |
fibroblast |
- |
36930, 696402, 711668 |
3.1.3.34 | deoxynucleotide 3'-phosphatase |
fibroblast |
- |
81083 |
3.4.21.41 | complement subcomponent C1r |
fibroblast |
- |
81402 |
3.4.21.42 | complement subcomponent C1s |
fibroblast |
skin and synovial membrane |
81402 |
3.4.24.11 | neprilysin |
fibroblast |
- |
735083, 81609, 81616 |
3.1.6.15 | N-sulfoglucosamine-3-sulfatase |
fibroblast |
- |
95166 |
3.4.11.1 | leucyl aminopeptidase |
fibroblast |
- |
95187 |
3.4.24.7 | interstitial collagenase |
fibroblast |
fibroblasts |
95823, 95825, 95832 |
3.1.1.13 | sterol esterase |
fibroblast |
- |
133841, 133842 |
3.1.3.2 | acid phosphatase |
fibroblast |
normal and mucolipidosis II fibroblasts |
134719 |
3.1.4.12 | sphingomyelin phosphodiesterase |
fibroblast |
- |
135151, 135152, 135154, 135186, 664929, 678142 |
3.1.4.40 | CMP-N-acylneuraminate phosphodiesterase |
fibroblast |
from skin of normal individuals and patients with salla disease |
135469 |
2.7.8.17 | UDP-N-acetylglucosamine-lysosomal-enzyme N-acetylglucosaminephosphotransferase |
fibroblast |
- |
135495, 645375, 671199 |
3.1.6.12 | N-acetylgalactosamine-4-sulfatase |
fibroblast |
in culture |
135563 |
3.1.6.8 | cerebroside-sulfatase |
fibroblast |
- |
135691, 653357 |
3.2.1.25 | beta-mannosidase |
fibroblast |
- |
136046 |
3.2.1.45 | glucosylceramidase |
fibroblast |
skin fibroblasts in culture |
136453, 136461, 136470, 136473, 136474, 136479, 136484, 136487 |
3.2.2.19 | [protein ADP-ribosylarginine] hydrolase |
fibroblast |
- |
137044 |
3.2.1.18 | exo-alpha-sialidase |
fibroblast |
- |
171114, 171144 |
3.2.1.33 | amylo-alpha-1,6-glucosidase |
fibroblast |
from skin biopsies |
171373 |
3.2.1.76 | L-iduronidase |
fibroblast |
- |
171551, 171553, 171554, 171559, 171562, 171569, 665552, 732511 |
3.5.1.12 | biotinidase |
fibroblast |
- |
171919, 171924, 171935, 670277 |
3.1.4.1 | phosphodiesterase I |
fibroblast |
tumor-derived and normal fibroblasts, patients with neurofibromatosis |
208373 |
3.2.1.49 | alpha-N-acetylgalactosaminidase |
fibroblast |
- |
208653 |
3.5.1.15 | aspartoacylase |
fibroblast |
- |
209008 |
3.5.2.9 | 5-oxoprolinase (ATP-hydrolysing) |
fibroblast |
- |
209384 |
3.6.1.1 | inorganic diphosphatase |
fibroblast |
cell culture |
209791 |
3.6.1.42 | guanosine-diphosphatase |
fibroblast |
- |
210055 |
5.6.1.1 | microtubule-severing ATPase |
fibroblast |
- |
210534, 210536, 210538, 210539, 699690, 700089 |
5.6.1.3 | plus-end-directed kinesin ATPase |
fibroblast |
- |
210541, 210544, 210550 |
3.6.4.6 | vesicle-fusing ATPase |
fibroblast |
from PBD patients |
210581, 210583 |
3.6.4.7 | peroxisome-assembly ATPase |
fibroblast |
- |
210588, 210589, 210590, 685048 |
7.6.2.1 | P-type phospholipid transporter |
fibroblast |
- |
246839, 668247 |
7.2.2.9 | P-type Cu2+ transporter |
fibroblast |
fibroblasts from the female Menkes translocation patient show no detectable expression of the transcript, five additional fibroblast lines from unrelated Menkes patients show alterations in the expression of this gene |
246908 |
1.1.1.153 | sepiapterin reductase (L-erythro-7,8-dihydrobiopterin forming) |
fibroblast |
amniotic, cell culture |
286016 |
1.1.1.153 | sepiapterin reductase (L-erythro-7,8-dihydrobiopterin forming) |
fibroblast |
dermal, cell culture |
286017 |
1.1.1.96 | diiodophenylpyruvate reductase |
fibroblast |
- |
287552 |
1.3.1.72 | DELTA24-sterol reductase |
fibroblast |
fibroblast from skin |
288497 |
3.10.1.1 | N-sulfoglucosamine sulfohydrolase |
fibroblast |
- |
288760 |
3.5.1.52 | peptide-N4-(N-acetyl-beta-glucosaminyl)asparagine amidase |
fibroblast |
C3H mouse-derived L-929 fibroblast cells |
288922, 288927 |
3.7.1.2 | fumarylacetoacetase |
fibroblast |
- |
289252 |
1.3.1.21 | 7-dehydrocholesterol reductase |
fibroblast |
- |
349276 |
1.3.3.4 | protoporphyrinogen oxidase |
fibroblast |
- |
390981 |
1.3.8.4 | isovaleryl-CoA dehydrogenase |
fibroblast |
- |
391206, 391212, 391214, 699381 |
1.3.8.6 | glutaryl-CoA dehydrogenase (ETF) |
fibroblast |
cultured |
391410 |
1.4.3.13 | protein-lysine 6-oxidase |
fibroblast |
- |
391742, 685981, 687597, 687919, 696449 |
1.5.1.15 | methylenetetrahydrofolate dehydrogenase (NAD+) |
fibroblast |
- |
392077, 392084, 392085, 392092, 671588 |
1.5.1.15 | methylenetetrahydrofolate dehydrogenase (NAD+) |
fibroblast |
embryo fibroblast |
392077 |
1.5.1.2 | pyrroline-5-carboxylate reductase |
fibroblast |
- |
392128 |
1.5.1.3 | dihydrofolate reductase |
fibroblast |
cultured 3T6 mouse embryo fibroblasts |
392270 |
1.6.5.4 | monodehydroascorbate reductase (NADH) |
fibroblast |
normal and virus-transformed |
392744 |
2.7.1.149 | 1-phosphatidylinositol-5-phosphate 4-kinase |
fibroblast |
- |
393080 |
3.2.1.52 | beta-N-acetylhexosaminidase |
fibroblast |
skin fibroblast |
393689 |
3.2.1.52 | beta-N-acetylhexosaminidase |
fibroblast |
- |
393690, 751281 |
1.8.5.1 | glutathione dehydrogenase (ascorbate) |
fibroblast |
- |
394019 |
1.3.8.8 | long-chain acyl-CoA dehydrogenase |
fibroblast |
- |
394665, 676027, 684281, 686160 |
1.13.11.52 | indoleamine 2,3-dioxygenase |
fibroblast |
diploid fibroblast cells FS-4 |
395503 |
1.3.1.22 | 3-oxo-5alpha-steroid 4-dehydrogenase (NADP+) |
fibroblast |
cultured |
396207 |
1.3.1.22 | 3-oxo-5alpha-steroid 4-dehydrogenase (NADP+) |
fibroblast |
cultured female and male genital skin fibroblasts |
396221 |
1.17.4.1 | ribonucleoside-diphosphate reductase |
fibroblast |
- |
437909 |
1.14.99.1 | prostaglandin-endoperoxide synthase |
fibroblast |
- |
438393 |
1.14.19.3 | acyl-CoA 6-desaturase |
fibroblast |
skin fibroblast |
438421 |
2.4.1.66 | procollagen glucosyltransferase |
fibroblast |
cultured skin fibroblasts |
439149 |
5.3.4.1 | protein disulfide-isomerase |
fibroblast |
cultured, confluent |
439186 |
1.14.11.2 | procollagen-proline 4-dioxygenase |
fibroblast |
- |
439213, 439242, 439243 |
1.14.11.2 | procollagen-proline 4-dioxygenase |
fibroblast |
adult and fetal skin, the type I enzyme is the main enzyme form |
439243 |
1.14.11.2 | procollagen-proline 4-dioxygenase |
fibroblast |
foreskin |
439247 |
1.5.1.34 | 6,7-dihydropteridine reductase |
fibroblast |
cell culture |
484975 |