1654:
421:
25:
961:. A lack of holocarboxylase synthetase activity may also alter the regulation of certain genes that are important for normal development. Researchers believe that these disruptions in important cellular functions lead to breathing problems, skin rashes, and the other characteristic signs and symptoms of holocarboxylase synthetase deficiency.
842:. In many of the body's tissues, holocarboxylase synthetase activates other specific enzymes (called biotin-dependent carboxylases) by attaching biotin to them. These carboxylases are involved in many critical cellular functions, including the production and breakdown of proteins, fats, and carbohydrates.
83:
Biotin-(propionyl-CoA-carboxylase (ATP-hydrolyzing)) ligasebiotin-propionyl-CoA-carboxylase (ATP-hydrolysing) ligasebiotin-[propionyl-CoA-carboxylase (ATP-hydrolyzing)] synthetasebiotin:apo-propanoyl-CoA:carbon-dioxide ligase (ADP-forming) ligase (AMP-forming)biotin-propionyl-CoA-carboxylase
910:
Holocarboxylase synthetase may also play a role in regulating the activity of genes. In the nucleus, the enzyme likely attaches biotin molecules to histones, which are structural proteins that bind to DNA and give chromosomes their shape. Changing the shape of histones may help determine whether
941:(a building block of proteins) for another amino acid in the holocarboxylase synthetase enzyme. Many of the known mutations occur in a region of the enzyme that binds to biotin. These mutations reduce the enzyme's ability to attach biotin to
870:
623:
84:(ATP-hydrolysing) synthetasepropionyl coenzyme A holocarboxylase synthetasepropionyl-CoA holocarboxylase synthetasebiotin-propionyl coenzyme A carboxylase synthetase
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in the HLCS gene have been identified in people with holocarboxylase synthetase deficiency. Most of these mutations substitute one
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985:"The enzymatic synthesis of propionyl coenzyme A holocarboxylase from d-biotinyl 5'-adenylate and the apocarboxylase"
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certain genes are turned on or off; however, it is not known how adding biotin affects gene regulation.
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1024:
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Holocarboxylase synthetase (biotin—(propionyl-Coenzyme A-carboxylase (ATP-hydrolysing)) ligase)
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1006:
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38:
Please expand the article to include this information. Further details may exist on the
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949:. Without biotin, carboxylases remain inactive and are unable to process
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biotin—(propionyl-Coenzyme A-carboxylase (ATP-hydrolysing)) ligase)
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826:). This enzyme is important for the effective use of
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428:It has been suggested that this article should be
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905:propionyl-CoA:carbon-dioxide ligase (ADP-forming)
874:propionyl-CoA:carbon-dioxide ligase (ADP-forming)
851:propionyl-CoA:carbon-dioxide ligase (ADP-forming)
914:The HLCS gene is located on the long (q) arm of
1705:
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8:
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983:Siegel L, Foote JL, Coon MJ (March 1965).
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609:
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1027:at the U.S. National Library of Medicine
1000:
857:
1649:
658:
459:
58:
1122:D-alanine—poly(phosphoribitol) ligase
931:Holocarboxylase synthetase deficiency
830:, a B vitamin found in foods such as
36:about EC 6.3.4.9, 6.3.4.11, 6.3.4.15.
7:
1670:
1668:
922:37,045,059 to base pair 37,284,372.
989:The Journal of Biological Chemistry
865:{\displaystyle \rightleftharpoons }
1684:. You can help Knowledge (XXG) by
1262:Von Hippel–Lindau tumor suppressor
332:
313:
289:
270:
248:
225:
201:
184:
160:
143:
14:
1652:
1228:Long-chain-fatty-acid—CoA ligase
419:
23:
1324:Carbamoyl phosphate synthetase
1218:Succinyl coenzyme A synthetase
1:
1002:10.1016/S0021-9258(18)97532-0
1746:Enzymes of unknown structure
1736:Genes on human chromosome 21
1025:holocarboxylase+synthetases
1767:
1667:
1309:Holocarboxylase synthetase
1304:Argininosuccinate synthase
1277:Anaphase-promoting complex
819:, is a family of enzymes (
809:Holocarboxylase synthetase
434:into a new article titled
62:Holocarboxylase synthetase
1530:Michaelis–Menten kinetics
1341:Glutamate–cysteine ligase
1299:Adenylosuccinate synthase
1092:Aminoacyl tRNA synthetase
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1422:Diffusion-limited enzyme
1029:Medical Subject Headings
845:The catalyzed reaction:
918:at position 22.1, from
891:, and ], whereas its 3
1680:-related article is a
1289:Glutathione synthetase
866:
34:is missing information
1515:Eadie–Hofstee diagram
1448:Allosteric regulation
1319:Asparagine synthetase
1223:Acetyl—CoA synthetase
867:
817:protein—biotin ligase
1525:Lineweaver–Burk plot
1247:Glutamine synthetase
872:AMP + diphosphate +
856:
883:of this enzyme are
849:ATP + biotin + apo-
1484:Enzyme superfamily
1417:Enzyme promiscuity
926:Related conditions
862:
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1240:: Carbon-Nitrogen
815:), also known as
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1520:Hanes–Woolf plot
1463:Enzyme activator
1458:Enzyme inhibitor
1432:Enzyme catalysis
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1252:Ubiquitin ligase
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310:RefSeq (protein)
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16:Class of enzymes
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1548:Oxidoreductases
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1510:Enzyme kinetics
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1494:List of enzymes
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1407:Catalytic triad
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1211:: Carbon-Sulfur
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1085:: Carbon-Oxygen
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1068:: CO CS and CN
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402:View/Edit Human
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353:Location (UCSC)
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1472:Classification
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1294:CTP synthetase
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1019:External links
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1016:
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995:(3): 1025–31.
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463:Biotin— ligase
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1489:Enzyme family
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1453:Cooperativity
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1412:Oxyanion hole
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1167:Phenylalanine
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981:
980:
976:
972:
971:Biotinylation
969:
968:
964:
962:
960:
959:carbohydrates
956:
952:
948:
944:
940:
936:
932:
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923:
921:
917:
916:chromosome 21
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594:Gene Ontology
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532:NiceZyme view
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449:November 2020
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267:RefSeq (mRNA)
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51:
48:November 2020
41:
35:
32:This article
30:
26:
21:
20:
1751:Ligase stubs
1686:expanding it
1672:
1664:
1626:Translocases
1623:
1610:
1597:
1584:
1571:
1561:Transferases
1558:
1545:
1402:Binding site
1314:GMP synthase
1308:
992:
988:
943:carboxylases
929:
913:
909:
878:
844:
816:
812:
808:
807:
765:
520:BRENDA entry
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233:
209:
192:
168:
151:
131:
126:
89:External IDs
45:
33:
1731:Human genes
1397:Active site
933:: About 30
901:diphosphate
787:Swiss-model
667:Identifiers
508:IntEnz view
491:37318-67-5
468:Identifiers
105:- orthologs
71:Identifiers
1725:Categories
1600:Isomerases
1574:Hydrolases
1441:Regulation
1187:Tryptophan
1162:Methionine
1147:Isoleucine
1107:Asparagine
977:References
939:amino acid
881:substrates
783:Structures
778:Search for
740:Other data
577:structures
544:KEGG entry
1479:EC number
1182:Threonine
1142:Histidine
1132:Glutamine
1127:Glutamate
1112:Aspartate
935:mutations
920:base pair
860:⇌
836:egg yolks
746:EC number
722:NM_000411
681:NCBI gene
497:Databases
117:Orthologs
94:GeneCards
40:talk page
1741:EC 6.3.4
1503:Kinetics
1427:Cofactor
1390:Activity
1192:Tyrosine
1117:Cysteine
1102:Arginine
1076:6.1-6.3)
1011:14284697
965:See also
951:proteins
947:histones
893:products
824:6.3.4.10
797:InterPro
751:6.3.4.10
648:proteins
636:articles
624:articles
581:RCSB PDB
479:6.3.4.10
391:Wikidata
1659:Biology
1613:Ligases
1383:Enzymes
1172:Proline
1152:Leucine
1137:Glycine
1097:Alanine
1070:ligases
1066:Enzymes
793:Domains
763:Chr. 21
729:UniProt
603:QuickGO
568:profile
551:MetaCyc
486:CAS no.
442:discuss
221:UniProt
180:Ensembl
123:Species
78:Aliases
1678:enzyme
1675:EC 6.3
1645:Portal
1587:Lyases
1257:Cullin
1197:Valine
1177:Serine
1157:Lysine
1031:(MeSH)
1009:
957:, and
903:, and
889:biotin
879:The 3
838:, and
828:biotin
734:P50747
717:RefSeq
710:609018
672:Symbol
631:PubMed
613:Search
599:AmiGO
587:PDBsum
527:ExPASy
515:BRENDA
503:IntEnz
474:EC no.
371:search
369:PubMed
139:Entrez
1673:This
1539:Types
1267:UBE3A
832:liver
767:q22.1
758:Locus
563:PRIAM
431:split
132:Mouse
127:Human
1682:stub
1631:list
1624:EC7
1618:list
1611:EC6
1605:list
1598:EC5
1592:list
1585:EC4
1579:list
1572:EC3
1566:list
1559:EC2
1553:list
1546:EC1
1282:UBR1
1272:Mdm2
1007:PMID
955:fats
945:and
895:are
840:milk
705:OMIM
698:4976
693:HGNC
686:3141
675:HLCS
643:NCBI
584:PDBe
539:KEGG
437:HLCS
1238:6.3
1209:6.2
1083:6.1
997:doi
993:240
897:AMP
885:ATP
619:PMC
575:PDB
444:)
440:. (
380:n/a
375:n/a
362:n/a
357:n/a
342:n/a
323:n/a
299:n/a
280:n/a
257:n/a
210:n/a
193:n/a
169:n/a
152:n/a
101:OMA
1727::
1334:II
1074:EC
1005:.
991:.
987:.
953:,
907:.
899:,
887:,
834:,
821:EC
601:/
99:;
96::
1713:e
1706:t
1699:v
1688:.
1647::
1633:)
1629:(
1620:)
1616:(
1607:)
1603:(
1594:)
1590:(
1581:)
1577:(
1568:)
1564:(
1555:)
1551:(
1375:e
1368:t
1361:v
1329:I
1072:(
1058:e
1051:t
1044:v
1013:.
999::
811:(
451:)
447:(
239:a
235:n
103::
50:)
46:(
42:.
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