KIZ

Chr 20AR

kizuna centrosomal protein

Also known as: C20orf19, HT013, Kizuna, NCRNA00153, PLK1S1, RP69

The protein encoded by this gene localizes to centrosomes, strengthening and stabilizing the pericentriolar region prior to spindle formation. The encoded protein usually remains with the mother centrosome after centrosomal duplication. Sevral transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Feb 2013]

Primary Disease Associations & Inheritance

Retinitis pigmentosa 69MIM #615780
AR
0
Active trials
9
Pubs (1 yr)
20
P/LP submissions
6%
P/LP missense
LOEUF
LOF
Mechanism· G2P
Clinical SummaryKIZ
🧬
Gene-Disease Validity (ClinGen)
KIZ-related retinopathy · ARDefinitive

Definitive — sufficient evidence for diagnostic panels

📋
ClinVar Variants
18 unique Pathogenic / Likely Pathogenic· 74 VUS of 200 total submissions
📖
GeneReview available — KIZ
Authoritative clinical overview · Recommended first read
Open GeneReview ↗

Population Genetics & Constraint

Constraint data not available from gnomAD.

Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
strongKIZ-related retinitis pigmentosaLOFAR

Predictions shown for reference only — model trained on dominant genes, not applicable to AR conditions.

DN
0.6260th %ile
GOF
0.4283th %ile
LOF
0.4234th %ile

The Badonyi & Marsh prediction model was trained exclusively on dominant disease genes. Predictions are not reliable for genes with autosomal recessive inheritance and are shown at reduced opacity for reference only.

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312. Mechanism ranking also informed by gnomAD constraint, ClinVar, and ClinGen data.

ClinVar Variant Classifications

200 submitted variants in ClinVar

Classification Summary

Pathogenic11
Likely Pathogenic7
VUS74
Likely Benign81
Benign6
Conflicting1
11
Pathogenic
7
Likely Pathogenic
74
VUS
81
Likely Benign
6
Benign
1
Conflicting

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
2
0
9
0
11
Likely Pathogenic
5
1
1
0
7
VUS
0
67
5
2
74
Likely Benign
0
5
38
38
81
Benign
2
0
4
0
6
Conflicting
1
Total9735740180

LoF = frameshift, stop gained/lost, canonical splice · Counts from ClinVar esearch · Updated hourly

View in ClinVar →

Protein Context — Lollipop Plot

KIZ · 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

No active trials found for this gene.

Search ClinicalTrials.gov →
Clinical Literature
Open Research Assistant →