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2018 4
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31 results

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MiR-612 regulates invadopodia of hepatocellular carcinoma by HADHA-mediated lipid reprogramming.
Liu Y, Lu LL, Wen D, Liu DL, Dong LL, Gao DM, Bian XY, Zhou J, Fan J, Wu WZ. Liu Y, et al. J Hematol Oncol. 2020 Feb 7;13(1):12. doi: 10.1186/s13045-019-0841-3. J Hematol Oncol. 2020. PMID: 32033570 Free PMC article.

SREBP2 protein expression level as well as its downstream targets, HMGCS1, HMGCR, MVD, SQLE were all deregulated by HADHA. Meanwhile, the ATP levels were reduced to 1/2 and 1/4 in HCCLM3(miR-612-OE) (p < 0.05) and HCCLM3(hadha-KD) (p < 0.01) respectively. More

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SREBP2 protein expression level as well as its downstream targets, HMGCS1, HMGCR, MVD, SQLE were all deregulated by HADHA. Meanwhile, …
HADHA overexpression disrupts lipid metabolism and inhibits tumor growth in clear cell renal cell carcinoma.
Liu S, Liu X, Wu F, Zhang X, Zhang H, Gao D, Bi D, Qu H, Ge J, Xu Y, Zhao Z. Liu S, et al. Exp Cell Res. 2019 Nov 1;384(1):111558. doi: 10.1016/j.yexcr.2019.111558. Epub 2019 Aug 28. Exp Cell Res. 2019. PMID: 31472118
Herein, the tumor inhibitory role of HADHA overexpression in ccRCC was investigated further. The quantitative proteomic analysis displayed that a total of 1293 and 1293 proteins were identified in HADHA overexpressed 786-O-hadha and vector-transfected control …
Herein, the tumor inhibitory role of HADHA overexpression in ccRCC was investigated further. The quantitative proteomic analysis disp …
Genetic diversity in Kashubs: the regional increase in the frequency of several disease-causing variants.
Jankowski M, Daca-Roszak P, Obracht-Prondzyński C, Płoski R, Lipska-Ziętkiewicz BS, Ziętkiewicz E. Jankowski M, et al. J Appl Genet. 2022 Dec;63(4):691-701. doi: 10.1007/s13353-022-00713-z. Epub 2022 Aug 15. J Appl Genet. 2022. PMID: 35971028 Free PMC article. Review.
These include the following: (1) c.662A > G (p.Asp221Gly) in LDLR, causing heterozygous familial hypercholesterolemia; (2) c.3700_3704del in BRCA1, associated with hereditary breast and ovarian cancer syndrome; (3) c.1528G > C (p.Glu510Gln) in HADHA, seen in long-cha …
These include the following: (1) c.662A > G (p.Asp221Gly) in LDLR, causing heterozygous familial hypercholesterolemia; (2) c.3700_3704del …
Defects in long-chain 3-hydroxy acyl-CoA dehydrogenase lead to hepatocellular carcinoma: A novel etiology of hepatocellular carcinoma.
Khare T, Khare S, Angdisen JJ, Zhang Q, Stuckel A, Mooney BP, Ridenhour SE, Gitan RS, Hammoud GM, Ibdah JA. Khare T, et al. Int J Cancer. 2020 Sep 1;147(5):1461-1473. doi: 10.1002/ijc.32943. Epub 2020 Mar 16. Int J Cancer. 2020. PMID: 32115688 Free article.
Here we report, for the first time, generation of a mouse model with a defect in long-chain 3-hydoxy acyl-CoA dehydrogenase (LCHAD). The LCHAD exon 15 deletion was embryonic lethal to the homozygous mice whereas heterozygous mice (HT) develop significant hepatic ste …
Here we report, for the first time, generation of a mouse model with a defect in long-chain 3-hydoxy acyl-CoA dehydrogenase (LCHAD). …
An Autopsy Analysis of a Patient With Long-Chain 3-Hydroxyacyl-CoA Dehydrogenase Deficiency Caused by Compound Heterozygous HADHA Gene Mutations.
Zhang Q, Yao N, Liu Z, Xu C, Ding Z. Zhang Q, et al. Am J Forensic Med Pathol. 2023 Dec 1;44(4):336-339. doi: 10.1097/PAF.0000000000000872. Epub 2023 Aug 5. Am J Forensic Med Pathol. 2023. PMID: 37549033
It is an autosomal recessive disease induced by a mutation in the HADHA gene, which encodes the LCHAD deficiency. The clinical manifestations of this disease are diverse, primarily affecting the heart, liver, and skeletal muscles. ...Of note, the mother had a histor …
It is an autosomal recessive disease induced by a mutation in the HADHA gene, which encodes the LCHAD deficiency. The clinical …
Analysis of a family with mitochondrial trifunctional protein deficiency caused by HADHA gene mutations.
Yang J, Yuan D, Tan X, Zeng Y, Tang N, Chen D, Tan J, Cai R, Huang J, Yan T. Yang J, et al. Mol Med Rep. 2022 Feb;25(2):47. doi: 10.3892/mmr.2021.12563. Epub 2021 Dec 8. Mol Med Rep. 2022. PMID: 34878152 Free PMC article.
The present study reported the clinical characteristics and compound heterozygous HADHA gene mutations of two patients with MTPD in the Chinese population. ...The present study may improve understanding of the HADHA gene mutation spectrum and clinical phenotype in t …
The present study reported the clinical characteristics and compound heterozygous HADHA gene mutations of two patients with MTPD in t …
Fatty acid beta oxidation enzyme HADHA is a novel potential therapeutic target in malignant lymphoma.
Yamamoto K, Abe S, Honda A, Hashimoto J, Aizawa Y, Ishibashi S, Takemura T, Hanagata N, Yamamoto M, Miura O, Kurata M, Kitagawa M. Yamamoto K, et al. Lab Invest. 2020 Mar;100(3):353-362. doi: 10.1038/s41374-019-0318-6. Epub 2019 Sep 16. Lab Invest. 2020. PMID: 31527828 Free article.
Moreover, downregulation of HADHA increased the susceptibility to doxorubicin (P = 0.002) and etoposide (P = 0.004). Moreover, these phenotypes were confirmed in an HADHA knockout system. Thus, we provide a basis for a novel therapeutic strategy through the regulati …
Moreover, downregulation of HADHA increased the susceptibility to doxorubicin (P = 0.002) and etoposide (P = 0.004). Moreover, these …
Deep geno- and phenotyping in two consanguineous families with CMT2 reveals HADHA as an unusual disease-causing gene and an intronic variant in GDAP1 as an unusual mutation.
Khani M, Taheri H, Shamshiri H, Moazzeni H, Hardy J, Bras JT, InanlooRahatloo K, Alavi A, Nafissi S, Elahi E. Khani M, et al. J Neurol. 2021 Feb;268(2):640-650. doi: 10.1007/s00415-020-10171-4. Epub 2020 Sep 8. J Neurol. 2021. PMID: 32897397
Sequence data, segregation analysis, bioinformatics analysis, evolutionary conservation, and information in the literature strongly implicated HADHA as the causative gene. An intronic variation positioned 23 nucleotides away from following intron/exon border in GDAP1 was u …
Sequence data, segregation analysis, bioinformatics analysis, evolutionary conservation, and information in the literature strongly implicat …
CLUH granules coordinate translation of mitochondrial proteins with mTORC1 signaling and mitophagy.
Pla-Martín D, Schatton D, Wiederstein JL, Marx MC, Khiati S, Krüger M, Rugarli EI. Pla-Martín D, et al. EMBO J. 2020 May 4;39(9):e102731. doi: 10.15252/embj.2019102731. Epub 2020 Mar 9. EMBO J. 2020. PMID: 32149416 Free PMC article.
Here, we show that in primary hepatocytes, CLUH coalesces in specific ribonucleoprotein particles that define the translational fate of target mRNAs, such as Pcx, Hadha, and Hmgcs2, to match nutrient availability. Moreover, CLUH granules play signaling roles, by recruiting …
Here, we show that in primary hepatocytes, CLUH coalesces in specific ribonucleoprotein particles that define the translational fate of targ …
31 results