GENE REFERENCE / HGNC:6742

LZTR1

leucine zipper like post translational regulator 1

protein-coding gene22q11.21Gene information

What does this gene do?

The LZTR1 gene provides instructions for making a protein whose exact function is unknown. The LZTR1 protein is made in cells throughout the body. Within cells, it is found in the Golgi apparatus, which is a structure in which newly produced proteins are modified. Studies suggest that the LZTR1 protein may help stabilize this structure. Researchers suspect that this protein may also be associated with the CUL3 ubiquitin ligase complex, which is part of the cell machinery that breaks down (degrades) unneeded proteins.

Based on its role in several tumor types, the LZTR1 protein is thought to act as a tumor suppressor. Tumor suppressors are proteins that keep cells from growing and dividing too rapidly or in an uncontrolled way.

Source: MedlinePlus, National Library of Medicine. Source publication: 2023-04-03. Source review: 2017-01.

ClinGen reviews

ClinGen reviews the evidence linking a particular gene to a condition. Each rating below describes the strength of that link. It does not classify a variant in your DNA file.

Condition	Inheritance	Evidence
Noonan syndromeMONDO:0018997 · Evaluated 2020-04-23	Autosomal dominant	DefinitiveRASopathy Gene Curation Expert Panel
Noonan syndromeMONDO:0018997 · Evaluated 2020-08-27	Autosomal recessive	DefinitiveRASopathy Gene Curation Expert Panel
LZTR1-related schwannomatosisMONDO:0014299 · Evaluated 2025-08-21	Autosomal dominant	DefinitiveHereditary Cancer Gene Curation Expert Panel

ClinVar records

These numbers show what has been submitted to ClinVar. They cannot tell you how likely you are to have a condition.

Submitted alleles: 5,213
Submissions for variants in or overlapping this gene: 9,650
Submissions explicitly reporting this gene: 9,169
Alleles reported pathogenic / likely pathogenic: 836
VariationIDs with uncertain significance: 2,422
VariationIDs with conflicting classifications: 293

The rows count different things: alleles, submissions or VariationIDs. Their categories can overlap, so do not add them together. Pathogenic/likely pathogenic counts exclude structural variants that merely overlap a gene. A dash in the source means “not reported,” not zero.

NCBI produces the gene-summary file each week using counts recorded at the start of the month. The numbers may differ from those on the live ClinVar website. Read the field definitions ↗

View this gene in ClinVar ↗

YOUR DNA FILE

Can your DNA file tell you about LZTR1?

From a DNA test kit (such as 23andMe, AncestryDNA or MyHeritage). These kits check a fixed list of about 600,000 to 700,000 positions across your whole genome. ClinVar lists 836 alleles in LZTR1 reported as pathogenic or likely pathogenic. A kit checks only some of these positions, and often none of them.

From whole genome sequencing (a VCF file). Sequencing reads most of your genome. Your file lists the places where your DNA differs from the reference genome. Some regions are hard to sequence, including parts of some genes that have near-identical copies elsewhere, and a VCF file does not record which positions were read well.

If a variant is not in your file, that does not mean you don’t have it.

What BioDecode checks

BioDecode compares every variant in your file with ClinVar, on your own computer. For each match, it shows the ClinVar classification, the review status and the condition it was reported for. Your file is not uploaded.

A ClinVar classification applies to one particular variant and the condition it was reported for. It does not tell you about other variants in LZTR1.

BioDecode cannot fill gaps in your test, diagnose a condition or replace clinical testing.

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