東北医科薬科大学　機能病態分子学教室

機能病態分子学教室		DIVISION OF GLYCOPATHOLOGY

〒981-8558 宮城県仙台市青葉区小松島4丁目4-1 東北医科薬科大学薬学部機能病態分子学教室分子生体膜研究所 TEL 022-727-0116 kinou＊tohoku-mpu.ac.jp （＊を@に変えてください）

教授/Professor	稲森啓一郎 (INAMORI, Kei-ichiro)

助教/Assistant Professor	新田昂大 (NITTA, Takahiro)

学部生・薬学科/Undergrads	6年生 4名，5年生 8名

Research interests

スフィンゴ糖脂質の生理的および病態生理的役割の解明
・スフィンゴ糖脂質の発現変化とメタボリックシンドロームや炎症性疾患における意義
・受容体シグナル伝達におけるスフィンゴ糖脂質の機能的役割
・マウスモデルを用いたスフィンゴ糖脂質の病態生理学的機能の解析
・スフィンゴ糖脂質欠損症の分子診断と病態解明につながる糖転移酵素遺伝子バリアントの機能解析

Elucidation of the physiological and pathophysiological roles of glycosphingolipids (GSLs)
・Altered expression of GSLs and their roles in metabolic syndrome and inflammatory diseases
・Functional roles of GSLs in receptor signaling
・Analysis of pathophysiological functions of GSLs using mouse models
・Functional analysis of variants of glycosyltransferase genes leading to molecular diagnosis and elucidation of the pathology of GSL deficiency

Publications

【2025】

・When ganglioside pathways go awry: congenital disorders and experimental insights.
Inamori KI, Inokuchi JI.
J Hum Genet. 2025; doi: 10.1038/s10038-025-01366-6.

・Metabolic and Structural Consequences of GM3 Synthase Deficiency: Insights from an HEK293-T Knockout Model.
Chiricozzi E, Lunghi G, Valsecchi M, Carsana EV, Bassi R, Di Biase E, Dobi D, Ciampa MG, Mauri L, Aureli M, Inamori KI, Inokuchi JI, Sonnino S, Fazzari M.
Biomedicines. 2025; 13(4):843. doi: 10.3390/biomedicines13040843.

・VarMeter2: An enhanced structure-based method for predicting pathogenic missense variants through Mahalanobis distance.
Ohno S, Ogura C, Yabuki A, Itoh K, Manabe N, Angata K, Togayachi A, Aoki-Kinoshita K, Furukawa JI, Inamori KI, Inokuchi JI, Kaname T, Nishihara S, Yamaguchi Y.
Comput Struct Biotechnol J. 2025; 27:1034-1047. doi: 10.1016/j.csbj.2025.02.008.

【2024】

・Functional evaluation of novel variants of B4GALNT1 in a patient with hereditary spastic paraplegia and the general population.
Inamori KI, Nakamura K, Shishido F, Hsu JC, Nagafuku M, Nitta T, Ikeda J, Yoshimura H, Kodaira M, Tsuchida N, Matsumoto N, Uemura S, Ohno S, Manabe N, Yamaguchi Y, Togayachi A, Aoki-Kinoshita KF, Nishihara S, Furukawa JI, Kaname T, Nakamura M, Shimohata T, Tadaka S, Shirota M, Kinoshita K, Nakamura Y, Ohno I, Sekijima Y, Inokuchi JI.
Front Neurosci. 2024; 18:1437668. doi: 10.3389/fnins.2024.1437668.

・Dietary gangliosides rescue GM3 synthase deficiency outcomes in mice accompanied by neurogenesis in the hippocampus.
Inokuchi JI, Go S, Suzuki A, Nakagawasai O, Odaira-Satoh T, Veillon L, Nitta T, McJarrow P, Kanoh H, Inamori KI, Tan-No K, Collett M.
Front Neurosci. 2024; 18:1387221. doi: 10.3389/fnins.2024.1387221.

・Perioperative changes in ganglioside monosialodihexosylganglioside (GM3) molecular species for benign prostatic hyperplasia: a preliminary report.
Anan G, Nitta T, Kanoh H, Sato M, Inokuchi JI.
Transl Androl Urol. 2024; 13(1):104-108. doi: 10.21037/tau-23-414.

・セラミド構造の多様性によるスフィンゴ糖脂質の機能制御
稲森啓一郎，狩野裕考，新田昂大，井ノ口仁一
生化学 2024; 96(4):448-456.

【2023】

・Chronic encephalomyelitis virus exhibits cellular tropism and evades pDCs by binding to sialylated integrins as the cell surface receptors.
Takeda K, Kaifu T, Michihata R, Kinugawa N, Fujioka A, Tateno A, Toshima K, Kanoh H, Inamori KI, Kamijo K, Himeda T, Ohara Y, Inokuchi JI, Nakamura A.
Eur J Immunol. 2023; 53(10):e2350452. doi: 10.1002/eji.202350452.

・Cell density-dependent membrane distribution of ganglioside GM3 in melanoma cells.
Murate M, Yokoyama N, Tomishige N, Richert L, Humbert N, Pollet B, Makino A, Kono N, Mauri L, Aoki J, Sako Y, Sonnino S, Komura N, Ando H, Kaneko MK, Kato Y, Inamori KI, Inokuchi JI, Mely Y, Iwabuchi K, Kobayashi T.
Cell Mol Life Sci. 2023 May 30;80(6):167. doi: 10.1007/s00018-023-04813-9.

・Neurological insights on two siblings with GM3 synthase deficiency due to novel compound heterozygous ST3GAL5 variants.
Watanabe S, Lei M, Nakagawa E, Takeshita E, Inamori KI, Shishido F, Sasaki M, Mitsuhashi S, Matsumoto N, Kimura Y, Iwasaki M, Takahashi Y, Mizusawa H, Migita O, Ohno I, Inokuchi JI.
Brain Dev. 2023; 45(5):270-277. doi: 10.1016/j.braindev.2023.01.002.

・Mass Spectrometry of Neutral Glycosphingolipids.
Suzuki A, Silsirivanit A, Watanabe T, Matsuda J, Inamori KI, Inokuchi JI.
Methods Mol Biol. 2023; 2613:127-144. doi: 10.1007/978-1-0716-2910-9_11.

・Sialyltransferase Activity Assay for Ganglioside GM3 Synthase.
Inamori KI, Nitta T, Shishido F, Watanabe S, Ohno I, Inokuchi JI.
Methods Mol Biol. 2023; 2613:101-110. doi: 10.1007/978-1-0716-2910-9_9.

【2022】

・GM3 synthase deficiency increases brain glucose metabolism in mice.
Bharathi SS, Zhang BB, Paul E, Zhang Y, Schmidt AV, Fowler B, Wu Y, Tiemeyer M, Inamori KI, Inokuchi JI, Goetzman ES.
Mol Genet Metab. 2022; 137(4):342-348. doi: 10.1016/j.ymgme.2022.10.006.

・Functional validation of novel variants in B4GALNT1 associated with early-onset complex hereditary spastic paraplegia with impaired ganglioside synthesis.
Alecu JE, Ohmi Y, Bhuiyan RH, Inamori KI, Nitta T, Saffari A, Jumo H, Ziegler M, de Gusmao CM, Sharma N, Ohno S, Manabe N, Yamaguchi Y, Kambe M, Furukawa K, Sahin M, Inokuchi JI, Furakawa K, Ebrahimi-Fakhari D.
Am J Med Genet A. 2022; 188(9):2590-2598. doi: 10.1002/ajmg.a.62880.

・Ganglioside GM3 Synthase Deficiency in Mouse Models and Human Patients.
Inamori KI, Inokuchi JI.
Int J Mol Sci. 2022; 23(10):5368. doi: 10.3390/ijms23105368.

・Regulation of Leptin Receptor Signaling by Gangliosides
Kei-ichiro Inamori
Trends Glycosci Glycotechnol. 2022; 34(198) E25-E28.

・Homeostatic and pathogenic roles of the GM3 ganglioside.
Inokuchi JI, Kanoh H, Inamori KI, Nagafuku M, Nitta T, Fukase K.
FEBS J. 2022; 289(17):5152-5165. doi: 10.1111/febs.16076.

【2021】

・GRASP55 regulates intra-Golgi localization of glycosylation enzymes to control glycosphingolipid biosynthesis.
Pothukuchi P, Agliarulo I, Pirozzi M, Rizzo R, Russo D, Turacchio G, Nuchel J, Yang JS, Gehin C, Capolupo L, Hernandez-Corbacho MJ, Biswas A, Vanacore G, Dathan N, Nitta T, Henklein P, Thattai M, Inokuchi JI, Hsu VW, Plomann M, Obeid LM, Hannun YA, Luini A, D'Angelo G, Parashuraman S.
EMBO J. 2021; 40(20):e107766. doi: 10.15252/embj.2021107766.

・Enzyme assay of GM3 synthase (lactosylceramide α2,3-sialyltransferase, GM3S, ST3GAL5).
Inamori KI, Inokuchi JI.
2021 Nov 11 [updated 2022 May 23]. In: Nishihara S, Angata K, Aoki-Kinoshita KF, Hirabayashi J, editors. Glycoscience Protocols (GlycoPODv2) [Internet]. Japan Consortium for Glycobiology and Glycotechnology; 2021-.

・Enzyme assay of N-acetylglucosaminyltransferase IX/Vb (MGAT5B).
Inamori KI, Maeda K, Taniguchi N.
2021 Oct 1 [updated 2022 Mar 15]. In: Nishihara S, Angata K, Aoki-Kinoshita KF, Hirabayashi J, editors. Glycoscience Protocols (GlycoPODv2) [Internet]. Japan Consortium for Glycobiology and Glycotechnology; 2021-.

・Enzyme assay of protein-O-mannosyl glycan glycosyltransferases (LARGE1/2).
Inamori KI.
2021 Oct 1 [updated 2022 Mar 15]. In: Nishihara S, Angata K, Aoki-Kinoshita KF, Hirabayashi J, editors. Glycoscience Protocols (GlycoPODv2) [Internet]. Japan Consortium for Glycobiology and Glycotechnology; 2021-.

・Structural characterization of glycosphingolipids by liquid chromatography-mass spectrometry.
Suzuki A, Nitta T, Silsirivanit A, Oikawa N, Matsuda J, Inokuchi JI.
2021 Sep 25 [updated 2022 Feb 27]. In: Nishihara S, Angata K, Aoki-Kinoshita KF, Hirabayashi J, editors. Glycoscience Protocols (GlycoPODv2) [Internet]. Japan Consortium for Glycobiology and Glycotechnology; 2021-.

・Relative content analysis of glycosphingolipids by liquid chromatography-mass spectrometry with multiple reaction monitoring.
Suzuki A, Nitta T, Silsirivanit A, Inokuchi JI.
2021 Sep 25 [updated 2022 Feb 27]. In: Nishihara S, Angata K, Aoki-Kinoshita KF, Hirabayashi J, editors. Glycoscience Protocols (GlycoPODv2) [Internet]. Japan Consortium for Glycobiology and Glycotechnology; 2021-.

【2020】

・Roles of Gangliosides in Hypothalamic Control of Energy Balance: New Insights.
Inamori KI, Inokuchi JI.
Int J Mol Sci. 2020; 21(15):5349. doi: 10.3390/ijms21155349.

・Homeostatic and pathogenic roles of GM3 ganglioside molecular species in TLR4 signaling in obesity.
Kanoh H, Nitta T, Go S, Inamori KI, Veillon L, Nihei W, Fujii M, Kabayama K, Shimoyama A, Fukase K, Ohto U, Shimizu T, Watanabe T, Shindo H, Aoki S, Sato K, Nagasaki M, Yatomi Y, Komura N, Ando H, Ishida H, Kiso M, Natori Y, Yoshimura Y, Zonca A, Cattaneo A, Letizia M, Ciampa M, Mauri L, Prinetti A, Sonnino S, Suzuki A, Inokuchi JI.
EMBO J. 2020; 39(12):e101732. doi: 10.15252/embj.2019101732.

【2019】

・Serum GM3(d18:1-16:0) and GM3(d18:1-24:1) levels may be associated with lymphoma: An exploratory study with haematological diseases.
Nishikawa M, Kurano M, Nitta T, Kanoh H, Inokuchi JI, Yatomi Y.
Sci Rep. 2019; 9(1):6308. doi: 10.1038/s41598-019-42817-3.

・Globo-series glycosphingolipids enhance Toll-like receptor 4-mediated inflammation and play a pathophysiological role in diabetic nephropathy.
Nitta T, Kanoh H, Inamori KI, Suzuki A, Takahashi T, Inokuchi JI.
Glycobiology. 2019; 29(3):260-268. doi: 10.1093/glycob/cwy105.

【2018】

・NPC1L1-dependent intestinal cholesterol absorption requires ganglioside GM3 in membrane microdomains.
Nihei W, Nagafuku M, Hayamizu H, Odagiri Y, Tamura Y, Kikuchi Y, Veillon L, Kanoh H, Inamori KI, Arai K, Kabayama K, Fukase K, Inokuchi JI.
J Lipid Res. 2018; 59(11):2181-2187. doi: 10.1194/jlr.M089201.

・Deficient ganglioside synthesis restores responsiveness to leptin and melanocortin signaling in obese KKAy mice.
Inamori KI, Ito H, Tamura Y, Nitta T, Yang X, Nihei W, Shishido F, Imazu S, Tsukita S, Yamada T, Katagiri H, Inokuchi JI.
J Lipid Res. 2018; 59(8):1472-1481. doi: 10.1194/jlr.M085753.

・Biology of GM3 Ganglioside.
Inokuchi JI, Inamori KI, Kabayama K, Nagafuku M, Uemura S, Go S, Suzuki A, Ohno I, Kanoh H, Shishido F.
Prog Mol Biol Transl Sci. 2018; 156:151-195. doi: 10.1016/bs.pmbts.2017.10.004.