General Information of the Ferroptosis Regulator (ID: REG10399)
Regulator Name Paired mesoderm homeobox protein 2 (PRRX2)
Synonyms
PMX2; PRX2; Paired-related homeobox protein 2
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Gene Name PRRX2
Gene ID 51450
Regulator Type Protein coding
Uniprot ID Q99811
Sequence
MDSAAAAFALDKPALGPGPPPPPPALGPGDCAQARKNFSVSHLLDLEEVAAAGRLAARPG
ARAEAREGAAREPSGGSSGSEAAPQDGECPSPGRGSAAKRKKKQRRNRTTFNSSQLQALE
RVFERTHYPDAFVREELARRVNLSEARVQVWFQNRRAKFRRNERAMLASRSASLLKSYSQ
EAAIEQPVAPRPTALSPDYLSWTASSPYSTVPPYSPGSSGPATPGVNMANSIASLRLKAK
EFSLHHSQVPTVN

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Family Paired homeobox family
Function
May play a role in the scarless healing of cutaneous wounds during the first two trimesters of development.

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HGNC ID
HGNC:21338
KEGG ID hsa:51450
Full List of the Ferroptosis Target of This Regulator and Corresponding Disease/Drug Response(s)
PRRX2 can regulate the following target(s), and cause disease/drug response(s). You can browse detail information of target(s) or disease/drug response(s).
Browse Target
Browse Disease
GTP cyclohydrolase 1 (GCH1) [Suppressor]
In total 1 item(s) under this target
Experiment 1 Reporting the Ferroptosis Target of This Regulator [1]
Target for Ferroptosis Suppressor
Responsed Disease Glioblastoma ICD-11: 2A00
Pathway Response Ferroptosis hsa04216
Cell Process Cell ferroptosis
Cell proliferation
In Vitro Model
GSC51 (WHO grade IV specimens)
GSC52 (WHO grade IV specimens)
GSC53 (WHO grade IV specimens)
GSC55 (WHO grade IV specimens)
GSC56 (WHO grade IV specimens)
GSC58 (WHO grade IV specimens)
In Vivo Model
Five-week-old female BALB/c nude mice were purchased from Shanghai Jihui Laboratory Animal Care Co., Ltd (Shanghai, China). All mice were bred in the Laboratory Animal Center of Shanghai Tenth Peoples Hospital under specific pathogen-free conditions. The animal experiments were performed by the Animal Care Committee of Shanghai Tenth Peoples Hospital. Briefly, each group contains five mice, and 5 x 104 GSCs were injected orthotopically into the mouse brain at 2 mm lateral and 2 mm anterior to the bregma with a stereotaxic apparatus. Then the survival time of each mouse was calculated, and the tumor volume was calculated according to the formula: V = (D x d2) / 2, where D represents the longest diameter and d represents the shortest diameter.

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Response regulation CircLRFN5 can be used as a potential Glioblastoma biomarker and become a target for molecular therapies or ferroptosis-dependent therapy in GBM. Mechanistically, circLRFN5 binds to PRRX2 protein and promotes its degradation via a ubiquitin-mediated proteasomal pathway. PRRX2 can transcriptionally upregulate GCH1 expression in GSCs, which is a ferroptosis suppressor via generating the antioxidant tetrahydrobiopterin (BH4).
Glioblastoma [ICD-11: 2A00]
In total 1 item(s) under this disease
Experiment 1 Reporting the Ferroptosis-centered Disease Response [1]
Target Regulator Paired mesoderm homeobox protein 2 (PRRX2) Protein coding
Pathway Response Ferroptosis hsa04216
Cell Process Cell ferroptosis
Cell proliferation
In Vitro Model
GSC51 (WHO grade IV specimens)
GSC52 (WHO grade IV specimens)
GSC53 (WHO grade IV specimens)
GSC55 (WHO grade IV specimens)
GSC56 (WHO grade IV specimens)
GSC58 (WHO grade IV specimens)
In Vivo Model
Five-week-old female BALB/c nude mice were purchased from Shanghai Jihui Laboratory Animal Care Co., Ltd (Shanghai, China). All mice were bred in the Laboratory Animal Center of Shanghai Tenth Peoples Hospital under specific pathogen-free conditions. The animal experiments were performed by the Animal Care Committee of Shanghai Tenth Peoples Hospital. Briefly, each group contains five mice, and 5 x 104 GSCs were injected orthotopically into the mouse brain at 2 mm lateral and 2 mm anterior to the bregma with a stereotaxic apparatus. Then the survival time of each mouse was calculated, and the tumor volume was calculated according to the formula: V = (D x d2) / 2, where D represents the longest diameter and d represents the shortest diameter.

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Response regulation CircLRFN5 can be used as a potential Glioblastoma biomarker and become a target for molecular therapies or ferroptosis-dependent therapy in GBM. Mechanistically, circLRFN5 binds to PRRX2 protein and promotes its degradation via a ubiquitin-mediated proteasomal pathway. PRRX2 can transcriptionally upregulate GCH1 expression in GSCs, which is a ferroptosis suppressor via generating the antioxidant tetrahydrobiopterin (BH4).
References
Ref 1 Correction: CircLRFN5 inhibits the progression of glioblastoma via PRRX2/GCH1 mediated ferroptosis. J Exp Clin Cancer Res. 2022 Nov 10;41(1):320. doi: 10.1186/s13046-022-02527-7.