Linear nevus sebaceous syndrome: genes and variants

Genes linked to Linear nevus sebaceous 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 disease-causing and 7 uncertain variants in HRAS are linked to Linear nevus sebaceous syndrome.

• 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 disease-causing and 2 uncertain variants in KRAS are linked to Linear nevus sebaceous syndrome.

• 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 disease-causing and 0 uncertain variants in NRAS are linked to Linear nevus sebaceous syndrome.

Known disease-causing variants in Linear nevus sebaceous syndrome

Same protein, different disease

• Costello syndrome is also caused by HRAS variants; they fall partly in the same places as the Linear nevus sebaceous syndrome variants (15 disease-causing).
• RASopathy is also caused by HRAS variants; they fall in the same places as the Linear nevus sebaceous syndrome variants (6 disease-causing).
• Large congenital melanocytic nevus is also caused by HRAS variants; they fall in the same places as the Linear nevus sebaceous syndrome variants (5 disease-causing).
• RASopathy is also caused by KRAS variants; they fall mostly in different places as the Linear nevus sebaceous syndrome variants (23 disease-causing).
• Noonan syndrome is also caused by KRAS variants; they fall mostly in different places as the Linear nevus sebaceous syndrome variants (16 disease-causing).
• Cardiofaciocutaneous syndrome is also caused by KRAS variants; they fall mostly in different places as the Linear nevus sebaceous syndrome variants (9 disease-causing).
• Autoimmune lymphoproliferative syndrome is also caused by KRAS variants; they fall in the same places as the Linear nevus sebaceous syndrome variants (5 disease-causing).
• Non-small cell lung carcinoma is also caused by KRAS variants; they fall mostly in different places as the Linear nevus sebaceous syndrome variants (5 disease-causing).
• Noonan syndrome is also caused by NRAS variants; they fall mostly in different places as the Linear nevus sebaceous syndrome variants (9 disease-causing).
• RASopathy is also caused by NRAS variants; they fall mostly in different places as the Linear nevus sebaceous syndrome variants (6 disease-causing).

Diseases related to Linear nevus sebaceous 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
• Malignant tumor of urinary bladder, also linked to HRAS and KRAS
• Costello syndrome, also linked to HRAS and NRAS
• Vascular malformation, also linked to KRAS and NRAS
• Juvenile myelomonocytic leukemia, also linked to KRAS and NRAS

Frequently asked questions

Which genes are linked to Linear nevus sebaceous syndrome?

In CATVariant, Linear nevus sebaceous syndrome is linked to 3 analyzed proteins: HRAS (GTPase HRas), KRAS (GTPase KRas) and NRAS (GTPase NRas).

How many genetic variants are linked to Linear nevus sebaceous syndrome?

20 variants: 5 are classified as disease-causing (pathogenic or likely pathogenic) in ClinVar and 9 are of uncertain significance or have conflicting reports.

Which uncertain variants in Linear nevus sebaceous syndrome look disease-causing?

None of the uncertain variants currently reaches the likely-pathogenic range on computable evidence alone.

About this data

Variant–disease links come from ClinVar, Open Targets and UniProt, pooled from the latest public CATVariant analysis 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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