Previous symbols: KRAS2. Aliases: KRAS1, K-Ras4B.
KRAS (KRas proto-oncogene, GTPase) is named by 43 disorder entries.
ai-gene-review gives KRAS's core molecular functions as GTP binding; and GTPase activity, acting in MAPK cascade; and Ras protein signal transduction. Unlike PTEN, here the KB and ai-gene-review use the same GO terms: the KB's KRAS nodes are annotated with MAPK cascade; and Ras protein signal transduction as well.
14 entries type KRAS as a somatic driver, among them Embryonal Rhabdomyosarcoma; Medullary Thyroid Carcinoma; and Pseudomyxoma Peritonei, as well as Endometriosis; and Brain arteriovenous malformation.
Pancreatic Ductal Adenocarcinoma records the gene less completely than its mechanism does. It places KRAS on the node KRAS Oncogene Activation with a functional impact of gain of function, but its genetic record is untyped. Nodes naming KRAS conform to the module sustaining_proliferative_signaling.
Three entries type KRAS as causative: Cardiofaciocutaneous Syndrome; Evans Syndrome; and Melorheostosis. Two of those record the variants as somatic, not germline: Evans Syndrome; and Melorheostosis. Cardiofaciocutaneous Syndrome's KRAS record gives no variant origin (unrecorded). ClinGen rates KRAS strong for cardiofaciocutaneous syndrome and definitive for Noonan syndrome, yet Noonan Syndrome's KRAS record is untyped.
Of the disorders that name KRAS,
19
have a genetic record with no relationship_type, and
5
name the gene with no genetic record at all. The entries ClinGen supports KRAS
for that do not type it are
Noonan Syndrome.
ClinGen also classifies KRAS for Costello syndrome, but as
disputed,
which is consistent with the Costello Syndrome entry not naming it.
Underlined values are claims re-computed from the knowledge base each time this page is built. The tables below are the source of truth.
KRAS encodes a membrane-associated Ras-family small GTPase that functions as a GDP/GTP molecular switch in receptor-driven signal transduction. In the GTP-bound state KRAS recruits effectors including RAF-family kinases and PI3Kalpha to activate downstream MAPK and PI3K-AKT-mTOR signaling; intrinsic and GAP-stimulated GTP hydrolysis return KRAS to the GDP-bound inactive state. KRAS4A and KRAS4B differ in their C-terminal hypervariable regions and membrane-targeting mechanisms, but both depend on lipid modification and membrane localization for signaling. Oncogenic and rasopathy variants alter nucleotide cycling, GAP responsiveness, effector interactions, or localization, producing context-dependent effects on proliferation, survival, senescence, and gene expression.
Relationship is the entry's genetic[].relationship_type. untyped means the entry has a genetic record for the gene but does not type it; its free-text association is shown instead.
| Disorder | Relationship | Where the gene appears |
|---|---|---|
| Adenomyosis | somatic driver |
mechanism: Somatic KRAS Activation in Endometrial Epithelium
|
| Ameloblastoma | somatic driver somatic |
mechanism: Somatic RTK-RAS-MAPK Driver Alterations
|
| Anal Canal Adenocarcinoma | biomarker somatic | |
| Appendiceal Neoplasm | somatic driver somatic | |
| Bile Duct Cyst | cooperating somatic | |
| Brain arteriovenous malformation | somatic driver somatic | |
| Cardiofaciocutaneous Syndrome | causative |
mechanism: RAS GTPase Dysregulation by Germline KRAS Variants
subtype: CFC2
scoped to subtype: CFC2
|
| Embryonal Rhabdomyosarcoma | somatic driver somatic |
mechanism: RAS Pathway Activation
|
| Endometriosis | somatic driver somatic | |
| Evans Syndrome | causative somatic |
mechanism: Loss of Lymphocyte Self-Tolerance
|
| Large Cell Neuroendocrine Carcinoma | somatic driver somatic |
mechanism: STK11 and KEAP1 Biallelic Inactivation
|
| Malignant Germ Cell Tumor of Ovary | somatic driver somatic |
mechanism: KIT and RAS-PI3K Oncogenic Signaling Activation
|
| Malignant Non-Dysgerminomatous Germ Cell Tumor Of Ovary | somatic driver somatic |
mechanism: Yolk Sac Tumor Molecular Alteration
|
| Medullary Thyroid Carcinoma | somatic driver somatic |
mechanism: RAS Mutation-Driven Proliferation
scoped to subtype: Sporadic MTC
|
| Melorheostosis | causative somatic | |
| Pseudomyxoma Peritonei | somatic driver somatic |
mechanism: KRAS and GNAS Driver Mutations in Appendiceal Mucinous Epithelium
|
| Rosai-Dorfman Disease | somatic driver somatic | |
| Thyroid Follicular Carcinoma | somatic driver somatic |
mechanism: RAS and PAX8-PPARG Driver Alterations
|
| Yolk Sac Tumor | somatic driver somatic |
scoped to subtype: Ovarian YST
|
| Central Nervous System Germ Cell Tumor |
untyped
Recurrent somatic KIT-RAS-pathway mutation
somatic
|
mechanism: Recurrent KIT-RAS Signaling Alterations
|
| Cervical Adenocarcinoma |
untyped
Somatic Mutation
|
|
| Colon Adenocarcinoma |
untyped
Somatic activating mutation
|
|
| Congenital Pulmonary Airway Malformation |
untyped
Pathogenic Variants
somatic
|
mechanism: Progression to Mucinous Adenocarcinoma; Somatic KRAS Mutations and Mucinous Cell Clusters
|
| Endometrial Endometrioid Adenocarcinoma |
untyped
Recurrent somatic RAS pathway driver alteration
somatic
|
|
| Ethmoid Sinus Adenocarcinoma |
untyped
Discordant somatic mutation frequency across series
|
|
| Gallbladder Cancer |
untyped
Somatic Gain-of-Function Mutation
|
|
| Glomus Tumor | untyped somatic |
mechanism: Constitutive Mitogenic Pathway Activation
|
| Juvenile Myelomonocytic Leukemia |
untyped
Somatic activating mutation
|
scoped to subtype: KRAS-mutated
|
| KRAS G12C-Mutant Non-Small Cell Lung Cancer |
untyped
Somatic Activating Mutation
|
|
| Mixed Germ Cell Tumor |
untyped
Copy-number gain in the majority of tumors; activating mutations in ~26% of cases
|
|
| Non-Small Cell Lung Cancer |
untyped
Activating Mutations
|
|
| Noonan Syndrome |
untyped
Pathogenic Variants
|
mechanism: Additional RAS-MAPK Signal-Amplifying Variants
subtype: NS3
scoped to subtype: NS3
|
| Ovarian Endometrioid Carcinoma |
untyped
Activating mutation
|
|
| Ovarian Mucinous Carcinoma |
untyped
Most frequent and earliest recurrent activating somatic driver mutation
|
|
| Pancreatic Ductal Adenocarcinoma |
untyped
Somatic Gain-of-Function Mutation
somatic
gain of function
|
mechanism: KRAS Oncogene Activation
|
| Pancreatic Mucinous Cystadenoma |
untyped
PREDISPOSING
|
|
| Testicular Germ Cell Tumor |
untyped
Activating mutations in ~14-26% of TGCTs
|
|
| Testicular Seminoma |
untyped
Copy-number gain (via 12p) very frequent; activating mutations recurrent
|
|
| BRAF V600E-Mutant Non-Small Cell Lung Cancer | no genetic record |
mechanism: MAPK Reactivation and Bypass Resistance
|
| Embryonal Carcinoma | no genetic record |
mechanism: Isochromosome 12p Formation and 12p Gain
|
| Endometrial Carcinoma | no genetic record |
mechanism: PTEN PI3K Pathway Dysregulation
|
| Plasma Cell Neoplasm | no genetic record |
mechanism: Precursor-to-Myeloma Clonal Progression
|
| Small Intestine Cancer | no genetic record |
mechanism: Adenoma-Carcinoma Sequence and Wnt-APC Activation
subtype: Adenocarcinoma
|
An entry matches when ClinGen's MONDO disease is the entry's own disease term, a subtype term, or an exact-match mapping. An amber status marks an entry ClinGen supports the gene for (Definitive, Strong or Moderate) whose own record does not type it. A Limited, Disputed or Refuted tier on such an entry is not a gap.
| ClinGen disease | MOI | Classification | dismech entry |
|---|---|---|---|
| Costello syndrome MONDO:0009026 | AD | Disputed 2018-07-24 |
Costello Syndrome
not named
|
| cardiofaciocutaneous syndrome MONDO:0015280 | AD | Strong 2018-07-24 |
Cardiofaciocutaneous Syndrome
causative
|
| Noonan syndrome MONDO:0018997 | AD | Definitive 2018-07-24 |
Noonan Syndrome
untyped
|
Identity from HGNC (snapshot 2026-10-01). Page layers are described in the gene pages documentation.