Linear nevus sebaceus syndrome: genes and variants

Genes linked to Linear nevus sebaceus syndrome

• HRAS: GTPase HRas

  Its GTP-bound state activates RAF-MEK-ERK and other pathways downstream of growth-factor receptors. Somatic activating variants drive several cancers, while germline activating variants cause Costello syndrome.

  2 ClinVar pathogenic / likely pathogenic and 7 uncertain variants in HRAS have source records linked to Linear nevus sebaceus syndrome. Association strength is not clinical gene validity.

• KRAS: GTPase KRas

  A small GTPase that acts as a molecular switch in the RAS-MAPK signaling pathway. By cycling between GDP- and GTP-bound states, it relays growth and survival signals, and activating KRAS variants are common drivers of cancer.

  2 ClinVar pathogenic / likely pathogenic and 2 uncertain variants in KRAS have source records linked to Linear nevus sebaceus syndrome. Association strength is not clinical gene validity.

• NRAS: GTPase NRas

  Its active GTP-bound state drives RAF-MEK-ERK and PI3K signaling downstream of growth-factor receptors. Somatic activating variants are common drivers of melanoma, leukemia, and other cancers, while germline activating variants can cause Noonan syndrome.

  1 ClinVar pathogenic / likely pathogenic and 0 uncertain variants in NRAS have source records linked to Linear nevus sebaceus syndrome. Association strength is not clinical gene validity.

ClinVar pathogenic and likely pathogenic variants linked to Linear nevus sebaceus syndrome

Same protein, different disease

• Costello syndrome also has ClinVar records linked to HRAS variants; they fall partly in the same places as the Linear nevus sebaceus syndrome variants (15 pathogenic / likely pathogenic).
• RASopathy also has ClinVar records linked to HRAS variants; they fall in the same places as the Linear nevus sebaceus syndrome variants (6 pathogenic / likely pathogenic).
• Large congenital melanocytic nevus also has ClinVar records linked to HRAS variants; they fall in the same places as the Linear nevus sebaceus syndrome variants (5 pathogenic / likely pathogenic).
• RASopathy also has ClinVar records linked to KRAS variants; they fall mostly in different places as the Linear nevus sebaceus syndrome variants (23 pathogenic / likely pathogenic).
• Noonan syndrome also has ClinVar records linked to KRAS variants; they fall mostly in different places as the Linear nevus sebaceus syndrome variants (16 pathogenic / likely pathogenic).
• Cardiofaciocutaneous syndrome also has ClinVar records linked to KRAS variants; they fall mostly in different places as the Linear nevus sebaceus syndrome variants (9 pathogenic / likely pathogenic).
• Autoimmune lymphoproliferative syndrome also has ClinVar records linked to KRAS variants; they fall in the same places as the Linear nevus sebaceus syndrome variants (5 pathogenic / likely pathogenic).
• Non-small cell lung carcinoma also has ClinVar records linked to KRAS variants; they fall mostly in different places as the Linear nevus sebaceus syndrome variants (5 pathogenic / likely pathogenic).
• Noonan syndrome also has ClinVar records linked to NRAS variants; they fall mostly in different places as the Linear nevus sebaceus syndrome variants (9 pathogenic / likely pathogenic).
• RASopathy also has ClinVar records linked to NRAS variants; they fall mostly in different places as the Linear nevus sebaceus syndrome variants (6 pathogenic / likely pathogenic).

Diseases related to Linear nevus sebaceus syndrome

• RASopathy, also linked to HRAS, KRAS and NRAS
• Noonan syndrome, also linked to HRAS, KRAS and NRAS
• Noonan syndrome and Noonan-related syndrome, also linked to HRAS, KRAS and NRAS
• Hypertrophic cardiomyopathy, also linked to HRAS and NRAS
• Cardiofaciocutaneous syndrome, also linked to KRAS and NRAS
• Acute myeloid leukemia, also linked to KRAS and NRAS
• Autoimmune lymphoproliferative syndrome, also linked to KRAS and NRAS
• Colorectal cancer, also linked to KRAS and NRAS
• Urinary bladder cancer, also linked to HRAS and KRAS
• Costello syndrome, also linked to HRAS and NRAS
• Vascular malformation, also linked to KRAS and NRAS
• Multiple myeloma, also linked to KRAS and NRAS

Frequently asked questions

Which genes have records linked to Linear nevus sebaceus syndrome?

This view contains 3 analyzed proteins: HRAS, KRAS, NRAS. Links come from clinical records and association databases. They do not imply that every listed gene is a validated cause, and missing genes may not yet be analyzed.

What do the clinical classifications mean?

Linked records include 5 pathogenic or likely pathogenic variants, 8 variants of uncertain significance and 1 with conflicting classifications. Labels summarize source records; multi-condition records may not make a separate assertion for this disease. Check the original record and review status.

Does the evidence score change a VUS classification?

No. 0 VUS or conflicting variants reach the likely-pathogenic points range on the computable criteria available here. This is a research prioritization signal, not a clinical classification. Patient, family and other required evidence may be missing.

Can I download the variant evidence?

Download the CSV for all 20 variants in the selected disease scope, including clinical labels, review status, evidence criteria, predictor scores, functional measurements and population frequency where available.

About this data

Variant–disease links come from ClinVar, Open Targets and UniProt, pooled from eligible public CATVariant analyses of each human protein. Evidence scores use the ACMG/AMP Bayesian points scale with computable criteria only (position among known disease variants, rarity in gnomAD, calibrated predictors, deep mutational scanning); there is no family or patient data, so they prioritise variants for expert review and never classify them.

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