TYR

Chr 11ADAR

tyrosinase

Also known as: ATN, CMM8, OCA1, OCA1A, OCAIA, SHEP3

The enzyme encoded by this gene catalyzes the first 2 steps, and at least 1 subsequent step, in the conversion of tyrosine to melanin. The enzyme has both tyrosine hydroxylase and dopa oxidase catalytic activities, and requires copper for function. Mutations in this gene result in oculocutaneous albinism, and nonpathologic polymorphisms result in skin pigmentation variation. The human genome contains a pseudogene similar to the 3' half of this gene. [provided by RefSeq, Oct 2008]

GeneReviewsOMIMResearchGenerating clinical summary…
LOFmechanismAD/ARLOEUF 1.965 OMIM phenotypes
Clinical SummaryTYR
🧬
Gene-Disease Validity (ClinGen)
oculocutaneous albinism type 1 · ARDefinitive

Definitive — sufficient evidence for diagnostic panels

Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
💊
Clinical Trials
12 active or recruiting trials — potential therapeutic options may be available
📖
GeneReview available — TYR
Authoritative clinical overview · Recommended first read
Open GeneReview ↗

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
1.96LOEUF
pLI 0.000
Z-score -3.29
OE 1.77 (1.331.96)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint?
-1.95Z-score
OE missense 1.32 (1.221.44)
379 obs / 286.1 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios?
LoF OE?1.77 (1.331.96)
00.351.4
Missense OE?1.32 (1.221.44)
00.61.4
Synonymous OE?1.30
01.21.6
LoF obs/exp: 38 / 21.5Missense obs/exp: 379 / 286.1Syn Z: -2.41

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

TYR · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

Carcinoma, Non-Small-Cell Lung

A Study of Participants in China With Non-Small-Cell Lung Cancer That is Unable to be Treated With Surgery

ACTIVE NOT RECRUITING
NCT05872763Hoffmann-La RocheStarted 2023-08-11
GIST - Gastrointestinal Stromal TumorKIT Gene Mutation

Comparing Regorafenib Combined With Envafolimab to Physician's Choice in Patients With Metastatic Gastrointestinal Stromal Tumors Harboring KIT Exon 17 Mutations Refractory to Standard Treatment

RECRUITING
NCT07559864Phase PHASE2Jian LiStarted 2025-07-14
physician-selected treatmentRegorafenib + Envafolimab
Acute Myeloid LeukemiaMyelodysplastic Syndrome With Excess Blasts-2

A Study of Gilteritinib Versus Midostaurin in Combination With Induction and Consolidation Therapy Followed by One-year Maintenance in Patients With Newly Diagnosed Acute Myeloid Leukemia or Myelodysplastic Syndromes With Excess Blasts-2 With FLT3 Mutations Eligible for Intensive Chemotherapy

ACTIVE NOT RECRUITING
NCT04027309Phase PHASE3Stichting Hemato-Oncologie voor Volwassenen NederlandStarted 2019-12-20
GilteritinibMidostaurin
Solid Tumors Harboring NTRK Fusion

A Study to Test the Safety and Efficacy of the Drug Larotrectinib for the Treatment of Tumors With NTRK-fusion in Children

ACTIVE NOT RECRUITING
NCT02637687Phase PHASE1, PHASE2BayerStarted 2015-12-16
Larotrectinib (Vitrakvi, BAY2757556)
Metastatic Digestive System Neuroendocrine NeoplasmMetastatic Neuroendocrine Tumor

Testing the Addition of an Anti-cancer Drug, Triapine, to the Usual Radiation-Based Treatment (Lutetium Lu 177 Dotatate) for Neuroendocrine Tumors

ACTIVE NOT RECRUITING
NCT04234568Phase PHASE1National Cancer Institute (NCI)Started 2020-07-20
Biospecimen CollectionComputed TomographyLutetium Lu 177 Dotatate
Chronic Myeloid Leukemia, Chronic PhaseAdult CMLLeukemia, Myeloid

Asciminib as Initial Therapy for Patients With Chronic Myeloid Leukemia in Chronic Phase

RECRUITING
NCT05143840Phase PHASE2University of Alabama at BirminghamStarted 2022-04-22
Single Agent AsciminibLow TKIElective Free Treatment
BRCA-Mutated Breast CarcinomaHER2-negative Breast Cancer

Neoadjuvant Treatment of gBRCA-Mutated HER2-Negative Breast Cancer With HRS-1167 and Famitinib ± Camrelizumab

RECRUITING
NCT06516289Phase PHASE2Fudan UniversityStarted 2024-09-30
HRS-1167FamitinibCamrelizumab
ALK Gene RearrangementALK Gene TranslocationALK Positive

Crizotinib in Treating Patients With Stage IB-IIIA Non-small Cell Lung Cancer That Has Been Removed by Surgery and ALK Fusion Mutations (An ALCHEMIST Treatment Trial)

ACTIVE NOT RECRUITING
NCT02201992Phase PHASE3ECOG-ACRIN Cancer Research GroupStarted 2015-03-23
Clinical ObservationCrizotinibLaboratory Biomarker Analysis
Carrier of PhenylketonuriaHealthy

Impact of Phenylalanine Elevations on Brain and Cognition in Adult PKU Carriers

RECRUITING
NCT07220265Phase NAUniversity of Missouri-ColumbiaStarted 2025-12-19
Phenylalanine (Phe)Placebo
Non-Small Cell Lung Cancer

Observational Prospective Study of Quality of Life in Unresectable TNM Stage III NSCLC (OBSTINATE)

ACTIVE NOT RECRUITING
NCT05049044Groupe Francais De Pneumo-CancerologieStarted 2020-12-18
Quality of Life Questionnaire-Core 30 (QLQ-C30)
Cancer Childhood

Ultrafast Whole Genome Sequencing for Childhood Cancer

RECRUITING
NCT07201038University of CambridgeStarted 2022-10-19
Acute Myeloid LeukemiaAML, AdultAML With Gene Mutations

Revumenib in Combination With 7+3 + Midostaurin in AML

RECRUITING
NCT06313437Phase PHASE1Richard Stone, MDStarted 2024-12-06
RevumenibMidostaurinCytarabine