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     See license terms at www.gsea-msigdb.org/gsea/license_terms_list. Please note that certain gene sets have special access terms.
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<MSIGDB BUILD_DATE="Jan 29, 2026" VERSION="2026.1.Hs" NAME="export">
    <GENESET STANDARD_NAME="ZHANG_FH_DEFICIENT_RCC_C1_VS_OTHERS_UP" SYSTEMATIC_NAME="M49272" HISTORICAL_NAMES="" PMID="40355427" AUTHORS="Zhang X,Zhao J,Yin X,Liang J,Wang Y,Zheng L,Tan P,Lin Y,Xu N,Zhu S,Chen J,Zhao J,Hu X,Pan X,Nie L,Zhang M,Chen Y,Zhang Y,Liu H,Dai J,Wang Z,Liu H,Ni Y,Rupp NJ,Moch H,Sheng X,Gong K,Liu X,Chen Z,He Z,Wang Y,Xu L,Liu M,Zhou H,Tang B,Huang R,Wei Q,Li X,Liu J,Yao J,Liao B,Liu Z,Shen P,Chen N,Zeng H,Sun G" GEOID="GSE273036" EXACT_SOURCE="Table S16, Genes_C1 vs. Others. Log2FC&gt;1;Padj&lt;0.01 41467_2025_59513_MOESM18_ESM" GENESET_LISTING_URL="" EXTERNAL_DETAILS_URL="https://www.nature.com/articles/s41467-025-59513-8" CHIP="Human_Ensembl_Gene_ID" CONTRIBUTOR="Anthony Castanza" CONTRIBUTOR_ORG="MSigDB Team" DESCRIPTION_BRIEF="Genes upregulated in the C1 subtype of FH-deficient RCC relative to C2 and C3 subtypes." DESCRIPTION_FULL="Fumarate hydratase-deficient renal cell carcinoma (FH-deficient RCC) is a rare yet highly lethal kidney cancer. To deepen understanding of FH-deficient RCC, the authors conduct a comprehensive integrated genomic study. The authors analyze the association of FH alteration patterns with tumor heterogeneity and develop a CpG site-specific methylation signature for precise identification of FH-deficient RCC. Transcriptomic analysis unveils three distinctive molecular subtypes characterized by enrichment of immune/Angiogenic/Stromal (C1), WNT/Notch/MAPK (C2), and proliferation/stemness (C3) pathways, respectively. Tumors in C1 derive the most substantial survival benefit from a combination of immune checkpoint blockade (ICB) and anti-angiogenic therapy. Tumors in C2 display moderate response to this therapeutic approach. In contrast, tumors in C3 exhibit an unfavorable response to anti-angiogenic monotherapy and its combination with ICB. These findings contribute to a profound understanding of the aggressive nature of FH-deficient RCC, offering insights into potential precision medicine approaches for disease management." TAGS="" MEMBERS="ENSG00000000971,ENSG00000004468,ENSG00000019169,ENSG00000025423,ENSG00000026751,ENSG00000029559,ENSG00000038945,ENSG00000043462,ENSG00000047457,ENSG00000049249,ENSG00000066294,ENSG00000070190,ENSG00000072041,ENSG00000075223,ENSG00000077274,ENSG00000078589,ENSG00000079257,ENSG00000080493,ENSG00000081237,ENSG00000082074,ENSG00000086061,ENSG00000086598,ENSG00000089012,ENSG00000090104,ENSG00000090382,ENSG00000096060,ENSG00000101307,ENSG00000101916,ENSG00000101938,ENSG00000102096,ENSG00000102524,ENSG00000102837,ENSG00000105366,ENSG00000105664,ENSG00000105967,ENSG00000106483,ENSG00000106560,ENSG00000106952,ENSG00000107798,ENSG00000109536,ENSG00000109943,ENSG00000110077,ENSG00000110079,ENSG00000110852,ENSG00000111341,ENSG00000111348,ENSG00000111897,ENSG00000112303,ENSG00000112335,ENSG00000112799,ENSG00000113088,ENSG00000113263,ENSG00000113790,ENSG00000114013,ENSG00000114200,ENSG00000115128,ENSG00000115165,ENSG00000115828,ENSG00000115919,ENSG00000115956,ENSG00000116824,ENSG00000117009,ENSG00000117091,ENSG00000117228,ENSG00000118785,ENSG00000118985,ENSG00000119917,ENSG00000121316,ENSG00000121933,ENSG00000122188,ENSG00000122862,ENSG00000123338,ENSG00000124491,ENSG00000125355,ENSG00000125730,ENSG00000125869,ENSG00000125870,ENSG00000126777,ENSG00000127920,ENSG00000130208,ENSG00000131711,ENSG00000132465,ENSG00000132541,ENSG00000133110,ENSG00000133321,ENSG00000133574,ENSG00000134028,ENSG00000134061,ENSG00000134242,ENSG00000134460,ENSG00000134827,ENSG00000135521,ENSG00000135697,ENSG00000135744,ENSG00000135838,ENSG00000136167,ENSG00000137033,ENSG00000137573,ENSG00000137575,ENSG00000137745,ENSG00000137752,ENSG00000137959,ENSG00000138182,ENSG00000138385,ENSG00000138722,ENSG00000138755,ENSG00000139329,ENSG00000140105,ENSG00000140379,ENSG00000140937,ENSG00000143119,ENSG00000143297,ENSG00000143387,ENSG00000143947,ENSG00000145423,ENSG00000145649,ENSG00000146070,ENSG00000146374,ENSG00000146386,ENSG00000147003,ENSG00000147138,ENSG00000147669,ENSG00000148677,ENSG00000150093,ENSG00000150337,ENSG00000152583,ENSG00000152952,ENSG00000153064,ENSG00000153140,ENSG00000153563,ENSG00000154269,ENSG00000154451,ENSG00000155304,ENSG00000155307,ENSG00000155659,ENSG00000155962,ENSG00000156234,ENSG00000156467,ENSG00000158869,ENSG00000160654,ENSG00000160791,ENSG00000161405,ENSG00000161970,ENSG00000162616,ENSG00000162645,ENSG00000162654,ENSG00000162692,ENSG00000162706,ENSG00000162739,ENSG00000163131,ENSG00000163376,ENSG00000163395,ENSG00000163519,ENSG00000163563,ENSG00000163564,ENSG00000163565,ENSG00000163584,ENSG00000163600,ENSG00000163606,ENSG00000163751,ENSG00000163823,ENSG00000164346,ENSG00000165168,ENSG00000166523,ENSG00000166710,ENSG00000167286,ENSG00000168421,ENSG00000168685,ENSG00000169245,ENSG00000169248,ENSG00000169413,ENSG00000169442,ENSG00000169908,ENSG00000170160,ENSG00000170312,ENSG00000170476,ENSG00000170571,ENSG00000171530,ENSG00000171659,ENSG00000171860,ENSG00000172116,ENSG00000172469,ENSG00000173391,ENSG00000174946,ENSG00000175857,ENSG00000176907,ENSG00000177409,ENSG00000178562,ENSG00000180964,ENSG00000181631,ENSG00000182326,ENSG00000182899,ENSG00000182985,ENSG00000183508,ENSG00000183542,ENSG00000183918,ENSG00000184293,ENSG00000185222,ENSG00000185862,ENSG00000187474,ENSG00000187955,ENSG00000188404,ENSG00000189057,ENSG00000196368,ENSG00000196611,ENSG00000196664,ENSG00000197614,ENSG00000197646,ENSG00000197747,ENSG00000198363,ENSG00000198763,ENSG00000198851,ENSG00000198886,ENSG00000198888,ENSG00000203747,ENSG00000204287,ENSG00000204397,ENSG00000204472,ENSG00000205413,ENSG00000213886,ENSG00000221963,ENSG00000231389,ENSG00000237541,ENSG00000237988,ENSG00000240065,ENSG00000241106,ENSG00000241343,ENSG00000254709,ENSG00000255819,ENSG00000260314,ENSG00000260802,ENSG00000271503,ENSG00000275385,ENSG00000280433" MEMBERS_SYMBOLIZED="ABRACL,ADAMDEC1,AGT,AIF1,ANKRD1,APOC1,APOL6,ARHGDIB,ASPH,B2M,BANK1,BCHE,BCL2A1,BCO1,C1S,C3,C3AR1,CADM1,CADM3,CAPN6,CARD16,CASP1,CCDC144A,CCL18,CCL5,CCR1,CCR5,CD180,CD2,CD200R1,CD28,CD38,CD3D,CD3E,CD3G,CD48,CD52,CD53,CD84,CD86,CD8A,CD8B,CDH11,CDK1,CETN3,CFH,CHRDL1,CLEC2B,CLEC4E,CLECL1P,CLIC2,CLTRN,COL14A1,COMP,CP,CPA3,CRTAM,CTSK,CTSS,CXCL10,CXCL11,CXCL13,CXCL9,CYBB,CYTIP,DAPP1,DNAJA1,DNAJB4,EHHADH,ELL2,EMB,ENPP3,ENSG00000280433,EVI2B,F13A1,FAM111B,FCER1G,FCGR1A,FCGR3A,FCRL5,FKBP5,FPR3,FRG1,FYB1,GAPT,GBP1,GBP2,GBP4,GBP5,GIMAP2,GIMAP4,GNG11,GPR171,GPR174,GPR34,GZMA,GZMK,HLA-DOB,HLA-DPA1,HLA-DQA2,HLA-DRA,HSD17B6,HSPA13,IBSP,ICOS,IFI16,IFI44L,IFIT3,IGFN1,IGLL5,IKZF3,IL2RA,IL33,IL7R,ITGB1,ITK,JCHAIN,KBTBD8,KIF20B,KLRC4,KLRC4-KLRK1,KMO,KTN1,KYNU,LAMP5,LAX1,LCP1,LCP2,LIPA,LTV1,LUM,LXN,LY86,LYZ,MANEA,MAP1B,MARCO,MFAP5,MGP,MMP1,MMP13,MMRN1,MNDA,MRC1,MS4A4A,MS4A6A,MSR1,MT-ND1,MT-ND2,MT-ND4,MZB1,NCKAP1L,NPL,NSA2,NUDT11,OLFM4,OLR1,OR2I1,P2RY10,P2RY13,PDCD1LG2,PIM2,PLA2G7,PLAAT4,PLBD1,PLEK,PLOD2,POLR2K,POSTN,PSMB9,PTPN22,PTPRC,PYHIN1,QPCT,RGS1,RHOH,RIDA,RNASE6,RPL22L1,RPL26,RPL35A,RPL36A,RPS27A,RSPO3,S100A10,SAMD9,SAMD9L,SAMSN1,SDCBP,SELL,SEMA3C,SERINC1,SERTM2,SF3B6,SFRP2,SFRP4,SH2D1A,SIGLEC8,SIRPB1,SIRPG,SLAMF6,SLAMF7,SLC4A4,SLC6A15,SNRPB2,SNX3,SPARCL1,SPP1,SRGN,SSB,SULF1,TBCA,TCEAL8,TCEAL9,TCIM,TCN1,TENT5C,TFEC,TLR7,TLR8,TM4SF1,TMED2,TMEM255A,TMIGD3,TNFRSF9,TNFSF13B,TNFSF8,TRAT1,UBD,UQCRB,VCAM1,VNN2,VSIG4,WARS1" MEMBERS_EZID="100423062,100528032,1009,10247,102724200,10326,10412,1043,10512,10537,10562,10563,10631,10673,1070,10959,10964,11040,11080,11326,114769,114836,115361,115362,1193,1230,1234,1311,131450,133418,1356,1359,149628,1513,1520,1536,160365,183,1962,199,200916,202309,2124,2162,219285,2207,2209,2214,22797,22806,2289,22915,22936,23213,2359,23705,24141,2483,2533,25797,26157,26191,26253,2633,2634,27063,27071,27181,27299,27334,2791,2857,29851,29909,3001,3003,3071,3075,3112,3113,3118,3122,3301,3381,341,3428,3437,3512,3559,3575,3604,3627,3688,3702,374393,3895,3936,3937,397,3988,399,401613,4060,4064,4068,4069,4071,4131,4256,4283,4312,4322,4332,4360,442197,444,4481,4535,4536,4538,4973,50852,51186,51237,51284,51311,51338,51639,5169,5341,5352,53630,53829,5440,54809,54855,54900,55024,55026,55117,55190,55303,55423,5552,56253,567,56892,56925,5698,57393,57413,57515,57823,57863,5788,58527,590,5920,597,5996,6039,6154,6165,6173,6233,6281,6352,6362,6373,6386,6402,64092,6423,64231,6424,6629,6696,6741,6782,6902,6947,716,718,719,7373,7381,7412,7453,7941,79694,79887,80380,8076,80830,80896,827,8302,834,83416,8404,84541,84636,84870,84946,8564,8630,8671,8685,8724,8832,8875,8942,90843,90865,91156,914,915,916,917,91851,925,926,940,942,944,9450,952,9585,9595,962,963,9720,983,9976" MEMBERS_MAPPING="ENSG00000000971,CFH,3075|ENSG00000004468,CD38,952|ENSG00000019169,MARCO,8685|ENSG00000025423,HSD17B6,8630|ENSG00000026751,SLAMF7,57823|ENSG00000029559,IBSP,3381|ENSG00000038945,MSR1,4481|ENSG00000043462,LCP2,3937|ENSG00000047457,CP,1356|ENSG00000049249,TNFRSF9,3604|ENSG00000066294,CD84,8832|ENSG00000070190,DAPP1,27071|ENSG00000072041,SLC6A15,55117|ENSG00000075223,SEMA3C,10512|ENSG00000077274,CAPN6,827|ENSG00000078589,P2RY10,27334|ENSG00000079257,LXN,56925|ENSG00000080493,SLC4A4,8671|ENSG00000081237,PTPRC,5788|ENSG00000082074,FYB1,2533|ENSG00000086061,DNAJA1,3301|ENSG00000086598,TMED2,10959|ENSG00000089012,SIRPG,55423|ENSG00000090104,RGS1,5996|ENSG00000090382,LYZ,4069|ENSG00000096060,FKBP5,2289|ENSG00000101307,SIRPB1,10326|ENSG00000101916,TLR8,51311|ENSG00000101938,CHRDL1,91851|ENSG00000102096,PIM2,11040|ENSG00000102524,TNFSF13B,10673|ENSG00000102837,OLFM4,10562|ENSG00000105366,SIGLEC8,27181|ENSG00000105664,COMP,1311|ENSG00000105967,TFEC,22797|ENSG00000106483,SFRP4,6424|ENSG00000106560,GIMAP2,26157|ENSG00000106952,TNFSF8,944|ENSG00000107798,LIPA,3988|ENSG00000109536,FRG1,2483|ENSG00000109943,CRTAM,56253|ENSG00000110077,MS4A6A,64231|ENSG00000110079,MS4A4A,51338|ENSG00000110852,CLEC2B,9976|ENSG00000111341,MGP,4256|ENSG00000111348,ARHGDIB,397|ENSG00000111897,SERINC1,57515|ENSG00000112303,VNN2,8875|ENSG00000112335,SNX3,8724|ENSG00000112799,LY86,9450|ENSG00000113088,GZMK,3003|ENSG00000113263,ITK,3702|ENSG00000113790,EHHADH,1962|ENSG00000114013,CD86,942|ENSG00000114200,BCHE,590|ENSG00000115128,SF3B6,51639|ENSG00000115165,CYTIP,9595|ENSG00000115828,QPCT,25797|ENSG00000115919,KYNU,8942|ENSG00000115956,PLEK,5341|ENSG00000116824,CD2,914|ENSG00000117009,KMO,8564|ENSG00000117091,CD48,962|ENSG00000117228,GBP1,2633|ENSG00000118785,SPP1,6696|ENSG00000118985,ELL2,22936|ENSG00000119917,IFIT3,3437|ENSG00000121316,PLBD1,79887|ENSG00000121933,TMIGD3,57413|ENSG00000122188,LAX1,54900|ENSG00000122862,SRGN,5552|ENSG00000123338,NCKAP1L,3071|ENSG00000124491,F13A1,2162|ENSG00000125355,TMEM255A,55026|ENSG00000125730,C3,718|ENSG00000125869,LAMP5,24141|ENSG00000125870,SNRPB2,6629|ENSG00000126777,KTN1,3895|ENSG00000127920,GNG11,2791|ENSG00000130208,APOC1,341|ENSG00000131711,MAP1B,4131|ENSG00000132465,JCHAIN,3512|ENSG00000132541,RIDA,10247|ENSG00000133110,POSTN,10631|ENSG00000133321,PLAAT4,5920|ENSG00000133574,GIMAP4,55303|ENSG00000134028,ADAMDEC1,27299|ENSG00000134061,CD180,4064|ENSG00000134242,PTPN22,26191|ENSG00000134460,IL2RA,3559|ENSG00000134827,TCN1,6947|ENSG00000135521,LTV1,84946|ENSG00000135697,BCO1,53630|ENSG00000135744,AGT,183|ENSG00000135838,NPL,80896|ENSG00000136167,LCP1,3936|ENSG00000137033,IL33,90865|ENSG00000137573,SULF1,23213|ENSG00000137575,SDCBP,6386|ENSG00000137745,MMP13,4322|ENSG00000137752,CASP1,834|ENSG00000137959,IFI44L,10964|ENSG00000138182,KIF20B,9585|ENSG00000138385,SSB,6741|ENSG00000138722,MMRN1,22915|ENSG00000138755,CXCL9,4283|ENSG00000139329,LUM,4060|ENSG00000140105,WARS1,7453|ENSG00000140379,BCL2A1,597|ENSG00000140937,CDH11,1009|ENSG00000143119,CD53,963|ENSG00000143297,FCRL5,83416|ENSG00000143387,CTSK,1513|ENSG00000143947,RPS27A,6233|ENSG00000145423,SFRP2,6423|ENSG00000145649,GZMA,3001|ENSG00000146070,PLA2G7,7941|ENSG00000146374,RSPO3,84870|ENSG00000146386,ABRACL,58527|ENSG00000147003,CLTRN,57393|ENSG00000147138,GPR174,84636|ENSG00000147669,POLR2K,5440|ENSG00000148677,ANKRD1,27063|ENSG00000150093,ITGB1,3688|ENSG00000150337,FCGR1A,2209|ENSG00000152583,SPARCL1,8404|ENSG00000152952,PLOD2,5352|ENSG00000153064,BANK1,55024|ENSG00000153140,CETN3,1070|ENSG00000153563,CD8A,925|ENSG00000154269,ENPP3,5169|ENSG00000154451,GBP5,115362|ENSG00000155304,HSPA13,6782|ENSG00000155307,SAMSN1,64092|ENSG00000155659,VSIG4,11326|ENSG00000155962,CLIC2,1193|ENSG00000156234,CXCL13,10563|ENSG00000156467,UQCRB,7381|ENSG00000158869,FCER1G,2207|ENSG00000160654,CD3G,917|ENSG00000160791,CCR5,1234|ENSG00000161405,IKZF3,22806|ENSG00000161970,RPL26,6154|ENSG00000162616,DNAJB4,11080|ENSG00000162645,GBP2,2634|ENSG00000162654,GBP4,115361|ENSG00000162692,VCAM1,7412|ENSG00000162706,CADM3,57863|ENSG00000162739,SLAMF6,114836|ENSG00000163131,CTSS,1520|ENSG00000163376,KBTBD8,84541|ENSG00000163395,IGFN1,91156|ENSG00000163519,TRAT1,50852|ENSG00000163563,MNDA,4332|ENSG00000163564,PYHIN1,149628|ENSG00000163565,IFI16,3428|ENSG00000163584,RPL22L1,200916|ENSG00000163600,ICOS,29851|ENSG00000163606,CD200R1,131450|ENSG00000163751,CPA3,1359|ENSG00000163823,CCR1,1230|ENSG00000164346,NSA2,10412|ENSG00000165168,CYBB,1536|ENSG00000166523,CLEC4E,26253|ENSG00000166710,B2M,567|ENSG00000167286,CD3D,915|ENSG00000168421,RHOH,399|ENSG00000168685,IL7R,3575|ENSG00000169245,CXCL10,3627|ENSG00000169248,CXCL11,6373|ENSG00000169413,RNASE6,6039|ENSG00000169442,CD52,1043|ENSG00000169908,TM4SF1,4071|ENSG00000170160,CCDC144A,9720|ENSG00000170312,CDK1,983|ENSG00000170476,MZB1,51237|ENSG00000170571,EMB,133418|ENSG00000171530,TBCA,6902|ENSG00000171659,GPR34,2857|ENSG00000171860,C3AR1,719|ENSG00000172116,CD8B,926|ENSG00000172469,MANEA,79694|ENSG00000173391,OLR1,4973|ENSG00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FILTERED_BY_SIMILARITY="" FOUNDER_NAMES="" REFINEMENT_DATASETS="" VALIDATION_DATASETS="" CATEGORY_CODE="C2" ORGANISM="Homo sapiens" SUB_CATEGORY_CODE="CGP"/>
</MSIGDB>
