The Conditions View presents the conditions you selected on a previous screen, and for each condition, the related Enzyme Commission code (when applicable), the Secretary's Advisory Committee on Heritable Disorders in Newborns and Children (SACHDNC) Category, and International Classification of Diseases, Ninth and Tenth Revisions, Clinical Modification (ICD-9-CM and ICD-10-CM) codes.
Condition1 | Abbreviation1 | SACHDNC Category2 | SNOMED CT Code3 | ICD-9- | ICD-10- | Affected Protein6 | EC#7 | UniProt Number8 | |||
---|---|---|---|---|---|---|---|---|---|---|---|
Hearing Loss | |||||||||||
Hearing Loss | HEAR | Core | 15188001 | 389.9 | H91.90 | N/A | N/A | N/A | |||
MS/MS (Tandem Mass Spectrometry) Measured Conditions | |||||||||||
Amino Acid Disorders | |||||||||||
Argininemia | ARG | Secondary | 23501004 | 270.6 | E72.21 | Arginase | 3.5.3.1 | P05089 P78540 | |||
Argininosuccinic aciduria | ASA | Core | 41013004 | 270.6 | E72.22 | Argininosuccinate lyase | 4.3.2.1 | P04424 | |||
Carbamoyl-phosphate synthase deficiency | CPS | Other | 62522004 | 270.6 | E72.29 | Carbamoyl-phosphate synthase (ammonia) | 6.3.4.16 | P31327 | |||
Citrullinemia type I | CIT-I | Core | 398680004 | 270.6 | E72.23 | Argininosuccinate synthase | 6.3.4.5 | P00966 | |||
Citrullinemia type II | CIT-II | Secondary | 30529005 | 270.6 | E72.23 | Calcium-binding mitochondrial carrier protein Aralar2 | None | Q9UJS0 | |||
Dihydrolipoamide dehydrogenase deficiency | E3 | Core | 29914000 | 270.3 | E71.0 | Dihydrolipoyl dehydrogenase | 1.8.1.4 | P09622 | |||
Disorders of biopterin biosynthesis | BIOPT-BS | Secondary | 237914002 | 270.1 | E70.1 | 6-pyruvoyltetrahydropterin synthase | 4.2.3.12 | Q03393 | |||
Disorders of biopterin regeneration | BIOPT-REG | Secondary | 58256000 | 270.1 | E70.1 | 6,7-dihydropteridine reductase | 1.5.1.34 | P09417 | |||
Girate atrophy of the retina | Hyper ORN | Other | 314467007 | 270.6 | H31.23 | Ornithine aminotransferase | 2.6.1.13 | P04181 | |||
Histidinemia | HIS | Other | 410058007 | 270.5 | E70.41 | Histidine ammonia-lyase | 4.3.1.3 | P42357 | |||
Homocystinuria | HCY | Core | 11282001 | 270.4 | E72.11 | Cystathionine beta-synthase | 4.2.1.22 | P35520 | |||
Homocystinuria-megaloblastic anemia | CBL G | Other | 237938003 | 270.4 | E72.11 | Methionine synthase | 2.1.1.13 | Q99707 | |||
Hydroxyprolinemia | OH PRO | Other | 25739007 | 270.8 | E72.59 | 4-oxoproline reductase | 1.1.1.104 | None | |||
Hyperlysinemia | Hyper LYS | Other | 58558003 | 270.7 | E72.3 | Saccharopine dehydrogenase (NADP(+), L-lysine-forming) | 1.5.1.8 | Q9UDR5 | |||
Hypermethioninemia | MET | Secondary | 43123004 | 270.4 | E72.19 | Methionine adenosyltransferase | 2.5.1.6 | P31153 Q00266 | |||
Hyperornithinemia-Hyperammonemia-Homocitrullinuria syndrome | HHH | Other | 30287008 | 270.6 | E72.4 | Mitochondrial ornithine transporter 1 | None | Q9Y619 | |||
Hyperphenylalaninemia (variant, benign) | H-PHE | Secondary | 68528007 | 270.1 | E70.1 | Phenylalanine 4-monooxygenase | 1.14.16.1 | P00439 | |||
Hyperprolinemia type I | PRO I | Other | 61071003 | 270.8 | E72.59 | Proline dehydrogenase | 1.5.99.8 | O43272 Q9UF12 | |||
Hyperprolinemia type II | PRO II | Other | 124177001 | 270.8 | E72.59 | 1-pyrroline-5-carboxylate dehydrogenase | 1.5.1.12 | P30038 | |||
Maple syrup urine disease | MSUD | Core | 27718001 | 270.3 | E71.0 | 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring) | 1.2.4.4 | P12694 P21953 | |||
Methylcobalamin deficiency | CBL E | Other | 360373000 | 270.4 | E72.11 | [Methionine synthase] reductase | 1.16.1.8 | Q9UBK8 | |||
Methylene tetrahydrofolate reductase deficiency | MTHFR | Other | 41797007 | 270.4 | E72.12 | Methylenetetrahydrofolate reductase (NAD(P)H) | 1.5.1.20 | P42898 | |||
Nonketotic hyperglycinemia (glycine encephalopathy) | NKHG | Other | 237939006 | 270.7 | E72.51 | Glycine cleavage system H protein, mitochondrial | None | P23434 | |||
Ornithine transcarbamylase deficiency | OTC | Other | 80908008 | 270.6 | E72.4 | Ornithine carbamoyltransferase | 2.1.3.3 | P00480 | |||
Phenylketonuria | PKU | Core | 7573000 | 270.1 | E70.0 | Phenylalanine 4-monooxygenase | 1.14.16.1 | P00439 | |||
Pyroglutamic acidemia | 5-OXO | Other | 39112005 | 282.2 | D55.1 | Glutathione synthase | 6.3.2.3 | P48637 | |||
Pyruvate carboxylase deficiency | PC | Other | 87694001 | 271.8 | E74.4 | Pyruvate carboxylase | 6.4.1.1 | P11498 | |||
Tyrosinemia type I | TYR-1 | Core | 410056006 | 270.2 | E70.21 | Fumarylacetoacetase | 3.7.1.2 | P16930 | |||
Tyrosinemia type II | TYR-II | Secondary | 4887000 | 270.2 | E70.21 | Tyrosine transaminase | 2.6.1.5 | P17735 | |||
Tyrosinemia type III | TYR-III | Secondary | 415764005 | 270.2 | E70.21 | 4-hydroxyphenylpyruvate dioxygenase | 1.13.11.27 | P32754 | |||
Valinemia | Hyper VAL | Other | 47719001 | 270.3 | E71.19 | Branched-chain-amino-acid transaminase | 2.6.1.42 | O15382 P54687 | |||
Fatty Acid Oxidation Disorders | |||||||||||
2,4-Dienoyl-CoA reductase deficiency | De-Red | Secondary | 444944006 | 277.85 | E71.318 | 2,4-dienoyl-CoA reductase (NADPH) | 1.3.1.34 | Q16698 Q9NUI1 | |||
Carnitine-acylcarnitine translocase deficiency | CACT | Secondary | 238003000 | 277.85 | E71.318 | Carnitine O-palmitoyltransferase | 2.3.1.21 | P23786 P50416 Q8TCG5 Q92523 | |||
Carnitine palmitoyltransferase I deficiency | CPT-Ia | Secondary | 238001003 | 277.85 | E71.318 | Carnitine O-palmitoyltransferase | 2.3.1.21 | P23786 P50416 Q8TCG5 Q92523 | |||
Carnitine palmitoyltransferase II deficiency | CPT-II | Secondary | 238002005 | 277.85 | E71.318 | Carnitine O-palmitoyltransferase | 2.3.1.21 | P23786 P50416 Q8TCG5 Q92523 | |||
Carnitine uptake defect | CUD | Core | 21764004 | 277.81 | E71.41 | Solute carrier family 22 member 5 | None | O76082 | |||
Glutaric acidemia type II | GA-2 | Secondary | 22886006 | 277.85 | E71.313 | Electron-transferring-flavoprotein dehydrogenase | 1.5.5.1 | Q16134 | |||
Long-chain L-3-Hydroxy acyl-CoA dehydrogenase deficiency | LCHAD | Core | 307127004 | 277.85 | E71.318 | Long-chain-3-hydroxyacyl-CoA dehydrogenase | 1.1.1.211 | P40939 | |||
Maternal carnitine uptake defect | CUD (mat) | Other | 206001006 | 760.9 | P00.89 | Solute carrier family 22 member 5 | None | O76082 | |||
Medium-chain acyl-CoA dehydrogenase deficiency | MCAD | Core | 128596003 | 277.85 | E71.311 | Acyl-CoA dehydrogenase | 1.3.99.3 | P11310 | |||
Medium-chain ketoacyl-CoA thiolase deficiency | MCKAT | Secondary | 124265004 | 277.85 | E71.318 | Acetyl-CoA C-acyltransferase | 2.3.1.16 | P09110 P42765 P55084 | |||
Short-chain acyl-CoA dehydrogenase deficiency | SCAD | Secondary | 124166007 | 277.85 | E71.312 | Butyryl-CoA dehydrogenase | 1.3.8.1 | P16219 | |||
Short-chain L-3-hydroxy acyl-CoA dehydrogenase deficiency | SCHAD | Secondary | 237998000 | 277.85 | E71.318 | 3-hydroxyacyl-CoA dehydrogenase | 1.1.1.35 | Q08426 Q16836 Q99714 | |||
Trifunctional protein deficiency | TFP | Core | 237999008 | 277.85 | E71.318 | Long-chain-3-hydroxyacyl-CoA dehydrogenase | 1.1.1.211 | P40939 | |||
Very long-chain acyl-CoA dehydrogenase deficiency | VLCAD | Core | 237997005 | 277.85 | E71.310 | Long-chain-acyl-CoA dehydrogenase | 1.3.99.13 | P28330 | |||
Organic Acid Disorders | |||||||||||
2-Methyl-3-hydroxybutyric aciduria | 2M3HBA | Secondary | 791000124107 | 270.3 | E71.19 | 3-hydroxy-2-methylbutyryl-CoA dehydrogenase | 1.1.1.178 | Q99714 | |||
2-Methylbutyrylglycinuria | 2MBG | Secondary | 445596006 | 270.3 | E71.19 | 3-hydroxyacyl-CoA dehydrogenase | 1.1.1.35 | Q99714 | |||
3-Hydroxy-3-methylglutaric aciduria | HMG | Core | 410059004 | 270.3 | E71.118 | Hydroxymethylglutaryl-CoA lyase | 4.1.3.4 | P35914 Q8TB92 | |||
3-Methylcrotonyl-CoA carboxylase deficiency | 3-MCC | Core | 13144005 | 270.3 | E71.19 | Methylcrotonoyl-CoA carboxylase | 6.4.1.4 | Q96RQ3 Q9HCC0 | |||
3-Methylglutaconic aciduria | 3MGA | Secondary | 297235006 | 270.3 | E71.111 | Methylglutaconyl-CoA hydratase | 4.2.1.18 | Q13825 | |||
beta-Ketothiolase deficiency | BKT | Core | 237953006 | 270.3 | E71.19 | Acetyl-CoA C-acyltransferase | 2.3.1.16 | P09110 P42765 P55084 | |||
Ethylmalonic encephalopathy | EMA | Other | 811000124106 | 277.87 | E88.49 | Electron-transferring-flavoprotein dehydrogenase | 1.5.5.1 | Q16134 | |||
Formiminoglutamic acidemia | FIGLU | Other | 59761008 | 270.5 | E70.49 | Glutamate formimidoyltransferase | 2.1.2.5 | O95954 | |||
Glutaric acidemia type I | GA-1 | Core | 76175005 | 270.7 | E72.3 | Glutaryl-CoA dehydrogenase | 1.3.99.7 | Q92947 | |||
Isobutyrylglycinuria | IBG | Secondary | 445571008 | 270.3 | E71.19 | 3-hydroxybutyryl-CoA dehydrogenase | 1.1.1.157 | None | |||
Isovaleric acidemia | IVA | Core | 87827003 | 270.3 | E71.110 | Isovaleryl-CoA dehydrogenase | 1.3.99.10 | P26440 | |||
Malonic acidemia | MAL | Secondary | 124594007 | 277.85 | E71.39 | Malonyl-CoA decarboxylase | 4.1.1.9 | O95822 | |||
Maternal 3-Methylcrotonyl-CoA carboxylase deficiency | 3-MCC (mat) | Other | 206001006 | 760.9 | P00.89 | Methylcrotonoyl-CoA carboxylase | 6.4.1.4 | Q96RQ3 Q9HCC0 | |||
Maternal glutaric acidemia type I | GA-1 (mat) | Other | 206001006 | 760.9 | P00.89 | Glutaryl-CoA dehydrogenase | 1.3.99.7 | Q92947 | |||
Methylmalonic acidemia | CBL A | Core | 73843004 | 270.3 | E71.120 | Methylmalonyl-CoA mutase | 5.4.99.2 | P22033 | |||
Methylmalonic acidemia | CBL B | Core | 82245003 | 270.3 | E71.120 | Methylmalonyl-CoA mutase | 5.4.99.2 | P22033 | |||
Methylmalonic acidemia | MUT | Core | 124680001 | 270.3 | E71.120 | Methylmalonyl-CoA mutase | 5.4.99.2 | P22033 | |||
Methylmalonic aciduria and homocystinuria | CBL C | Secondary | 74653006 | 270.3 | E71.120 | Methylmalonyl-CoA mutase | 5.4.99.2 | P22033 | |||
Methylmalonic aciduria and homocystinuria | CBL D | Secondary | 31220004 | 270.3 | E71.120 | Methylmalonic aciduria and homocystinuria type D protein, mitochondrial | None | Q9H3L0 | |||
Multiple carboxylase deficiency | MCD | Core | 360369003 | 270.3 | D81.818 | Biotin--[methylcrotonoyl-CoA-carboxylase] ligase | 6.3.4.11 | P50747 | |||
Primary lactic acidemia (various types) | LACTIC | Other | 190882007 | 276.2 | E87.2 | various enzymes | N/A | N/A | |||
Propionic acidemia | PROP | Core | 69080001 | 270.3 | E71.121 | Propionyl-CoA carboxylase | 6.4.1.3 | P05165 P05166 | |||
Succinyl-CoA ligase deficiency | SUCLA2 | Other | 445275003 | 277.87 | E88.40 | Succinate--CoA ligase (ADP-forming) | 6.2.1.5 | Q9P2R7 | |||
Non MS/MS Measured Conditions | |||||||||||
Cystic Fibrosis | |||||||||||
Cystic fibrosis | CF | Core | 190905008 | 277.00 | E84 | Cystic fibrosis transmembrane conductance regulator | N/A | P13569 | |||
Endocrine Disorders | |||||||||||
Adrenal Hyperplasia | |||||||||||
Congenital Adrenal Hyperplasia (11-beta monooxygenase) | CAH (11B-OHD) | Other | 124214007 | 255.2 | E25.0 | Steroid 11-beta-monooxygenase | 1.14.15.4 | P15538 P19099 | |||
Congenital Adrenal Hyperplasia (non-classical) | CAH (NC) | Core | 237754008 | 255.2 | E25.0 | Steroid 21-monooxygenase | 1.14.99.10 | P08686 | |||
Congenital Adrenal Hyperplasia (salt-wasting) | CAH (SW) | Core | 71578002 | 255.2 | E25.0 | Steroid 21-monooxygenase | 1.14.99.10 | P08686 | |||
Congenital Adrenal Hyperplasia (simple virilizing) | CAH (SV) | Core | 52604008 | 255.2 | E25.0 | Steroid 21-monooxygenase | 1.14.99.10 | P08686 | |||
Thyroid Disorders | |||||||||||
Primary Congenital Hypothyroidism | CH | Core | 190268003 | 243 | E03.1 | Thyrotropin receptor | N/A | P16473 | |||
Secondary Congenital Hypothyroidism | CH2 | Other | 82598004 | 243 | E03.1 | Thyrotropin subunit beta | N/A | P01222 | |||
Thyroid-Binding Globulin Deficiency | TBG | Other | 237544006 | 246.8 | E07.89 | Thyrotropin subunit beta TSHR protein | N/A | P01222 Q0VAP8 | |||
Hemoglobin Disorders | |||||||||||
Hemoglobinopathies | |||||||||||
Hb beta zero-thalassemia | Hb F only | Secondary | 86715000 | 282.49 | D56.1 | Hemoglobin subunit beta | N/A | P68871 | |||
Hb C beta-thalassemia | Hb F,C,A | Secondary | 61777009 | 282.49 | D56.9 | Hemoglobin subunit beta | N/A | P68871 | |||
Hb C-disease | Hb F,C | Secondary | 51053007 | 282.7 | D58.2 | Hemoglobin subunit beta | N/A | P68871 | |||
Hb D beta-thalassemia | Hb F,D,A | Secondary | 47047009 | 282.49 | D56.9 | Hemoglobin subunit beta | N/A | P68871 | |||
Hb disease other than A,C,D,E,F,H,O-Arab,S | Hb F, and other than A,C,D,E,F,H,O-Arab,S | Secondary | 80141007 | 282.7 | D58.2 | Hemoglobin subunit beta | N/A | P68871 | |||
Hb E beta-thalassemia | Hb F,E,A | Secondary | 234392002 | 282.49 | D56.9 | Hemoglobin subunit beta | N/A | P68871 | |||
Hb E-disease | Hb F,E | Secondary | 25065001 | 282.7 | D58.2 | Hemoglobin subunit beta | N/A | P68871 | |||
Hb H-disease | Hb F,H | Secondary | 48553001 | 282.49 | D56.0 | Hemoglobin subunit beta | N/A | P68871 | |||
Hb S beta-thalassemia | Hb F,S,A | Core | 127041004 | 282.41 | D57.40 | Hemoglobin subunit beta | N/A | P68871 | |||
Hb SC-disease | Hb F,S,C | Core | 35434009 | 282.63 | D57.20 | Hemoglobin subunit beta | N/A | P68871 | |||
Hb SD-disease | Hb F,S,D | Secondary | 25472008 | 282.68 | D57.80 | Hemoglobin subunit beta | N/A | P68871 | |||
Hb SE-disease | Hb F,S,E | Secondary | 47024008 | 282.68 | D57.80 | Hemoglobin subunit beta | N/A | P68871 | |||
Hb S O-Arab disease | Hb F,S,O-Arab | Secondary | 127048005 | 282.68 | D57.80 | Hemoglobin subunit beta | N/A | P68871 | |||
Hb S Other than A,C,D,E,O-Arab | Hb F,S and other than A,C,D,E,O-Arab | Secondary | 23269001 | 282.68 | D57.80 | Hemoglobin subunit beta | N/A | P68871 | |||
Hb SS-disease (sickle cell anemia) | Hb F,S | Core | 127040003 | 282.60 | D57.1 | Hemoglobin subunit beta | N/A | P68871 | |||
Hemoglobinopathy Traits | |||||||||||
Hb carrier other than C,D,E,S,O-Arab | Hb F,A and other than C,D,E,S,O-Arab | Secondary | 123773003 | 282.7 | D58.2 | Hemoglobin subunit beta | N/A | P68871 | |||
Hb C-carrier | Hb F,A,C | Secondary | 76050008 | 282.7 | D58.2 | Hemoglobin subunit beta | N/A | P68871 | |||
Hb D-carrier | Hb F,A,D | Secondary | 7391009 | 282.7 | D58.2 | Hemoglobin subunit beta | N/A | P68871 | |||
Hb E-carrier | Hb F,A,E | Secondary | 46248003 | 282.7 | D58.2 | Hemoglobin subunit beta | N/A | P68871 | |||
Hb O-Arab carrier | Hb F,A,O-Arab | Secondary | None | 282.7 | D58.2 | Hemoglobin subunit beta | N/A | P68871 | |||
Hb S (sickle)-carrier | Hb F,A,S | Secondary | 16402000 | 282.5 | D57.3 | Hemoglobin subunit beta | N/A | P68871 | |||
Infectious Diseases | |||||||||||
Congenital toxoplasmosis | TOXO | Other | 73893000 | 771.2 | P37.1 | N/A | N/A | N/A | |||
Human immunodeficiency virus | HIV | Other | 52079000 | 042 | B20 | N/A | N/A | N/A | |||
Biotinidase | |||||||||||
Biotinidase Deficiency | BIO | Core | 8808004 | 277.6 | D81.810 | Biotinidase | 3.5.1.12 | P43251 | |||
Galactose Disorders | |||||||||||
Classical galactosemia (galactose-1-phosphate uridyltransferase deficiency) | GALT | Core | 398664009 | 271.1 | E74.21 | UDP-glucose--hexose-1-phosphate uridylyltransferase | 2.7.7.12 | P07902 | |||
Galactoepimerase deficiency (uridine diphosphate galactose 4-epimerase deficiency) | GALE | Secondary | 8849004 | 271.1 | E74.21 | UDP-glucose 4-epimerase | 5.1.3.2 | Q14376 | |||
Galactokinase deficiency | GALK | Secondary | 124302001 | 271.1 | E74.29 | Galactokinase | 2.7.1.6 | P51570 | |||
Lysosomal Storage Disorders | |||||||||||
Fabry disease | GLA | Other | 16652001 | 272.7 | E75.21 | Alpha-galactosidase | 3.2.1.22 | P06280 | |||
Gaucher disease | GBA | Other | 190794006 | 272.7 | E75.22 | Glucosylceramidase | 3.2.1.45 | P04062 | |||
Krabbe disease | GALC | Other | 192782005 | 330.0 | E75.23 | Galactosylceramidase | 3.2.1.46 | P54803 | |||
Niemann Pick disease A/B | ASM | Other | 58459009 | 272.7 | E75.249 | Sphingomyelin phosphodiesterase | 3.1.4.12 | P17405 | |||
Pompe disease | GAA | Other | 237967002 | 271.0 | E74.02 | Alpha-glucosidase | 3.2.1.20 | P10253 | |||
Other Disorders | |||||||||||
Glucose-6-phosphate dehydrogenase deficiency | G6PD | Other | 62403005 | 282.2 | D55.0 | Glucose-6-phosphate dehydrogenase | 1.1.1.49 | P11413 | |||
Severe combined immunodeficiency | SCID | Core | 31323000 | 279.2 | D81.9 | N/A | N/A | N/A |
The above view reflects the criteria you selected on the previous screen.
Legend
Condition Name and Abbreviation — curated by the NLM and selected from among the names used by the Secretary's Advisory Committee on Heritable Disorders in Newborns and Children (SACHDNC), National Newborn Screening Information System (NNSIS), the American College of Medical Genetics (ACMG), the HHS Office of the National Coordinator for Health Information Technology (ONC)/American Health Information Community (AHIC) Personalized Health Care Work Group, and input from the newborn screening community.
SACHDNC Category — based on the U.S. Department of Health and Human Services (HHS) Recommended Uniform Screening Panel of the Secretary's Advisory Committee on Heritable Disorders in Newborns and Children (SACHDNC). Conditions designated as "core" by SACHDNC should be included in every newborn screening program, and "secondary" conditions are some of the disorders that may be detected during screening for a core disorder. Conditions classified as "other" are those that are screened for by some states but are not part of the Recommended Uniform Screening Panel.
SNOMED CT® Code — Systematized Nomenclature of Medicine — Clinical Terms code is assigned by the International Health Terminology Standards Development Organisation (IHTSDO). SNOMED CT is a concept-oriented clinical terminology that has been designated as a U.S. standard for electronic health information exchange. The Newborn Screening Coding and Terminology Guide uses some codes from the US Extension to SNOMED CT.
ICD-9-CM Code — International Classification of Diseases, Ninth Revision, Clinical Modification code is assigned to diagnoses associated with hospital utilization in the U.S. It is a current US standard for use in administrative healthcare transactions. Although ICD-9-CM codes are fairly specific, in certain cases, the same ICD-9-CM code might apply to several disorders in the same group (e.g. amino acid disorders).
ICD-10-CM Code — International Classification of Diseases, Tenth Revision, Clinical Modification code will be replacing ICD-9-CM codes in 2013 as a HIPAA standard. Although ICD-10-CM codes are fairly specific, in certain cases, the same ICD-10-CM code might apply to multiple related disorders.
Affected Protein — the structure that is abnormal in this condition. The affected protein could be an enzyme, a hormone, or a specific molecule such as hemoglobin or immunoglobulin chain. If the affected protein is an enzyme, it will have an Enzyme Commission (EC) number.
Enzyme Commission (EC) Number — a unique identifier for the affected enzyme (if the affected protein is an enzyme); the EC number is assigned by the Recommendations of the Nomenclature Committee of the International Union of Biochemistry and Molecular Biology on the Nomenclature and Classification of Enzymes by the Reactions they Catalyse.
UniProt Number — a unique identifier assigned to all proteins, including enzymes, hemoglobin subunits, and immunoglobulin chains. The UniProt database is maintained by the Universal Protein Resource, an international collaboration.