title: The reduced expression of proximal tubular transporters in acquired Fanconi syndrome with κ light chain deposition.
abstract: In a case of acquired Fanconi syndrome associated with smoldering myeloma, we confirmed the deposition of protease-resistant κ light chain proteins in a proximal tubular injury and found the decreased expression of apical tubular transporters including sodium glucose co-transporter, sodium phosphate co-transporter, uric acid transporter 1, and a decrease of Na(+)/K(+)-ATPase in the basolateral membrane. The protease-resistant kappa light chain has a pathological role in the expression of tubular transporters in the proximal tubule and causes Fanconi syndrome associated with smoldering myeloma.
authors.affiliation: Division of Nephrology and Endocrinology, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-8655, Japan. akitojo-tky@umin.ac.jp.
authors.forename: Akihiro
authors.initials: A
authors.lastname: Tojo
authors.affiliation: Division of Nephrology and Endocrinology, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-8655, Japan.
authors.forename: Kensuke
authors.initials: K
authors.lastname: Asaba
authors.affiliation: Division of Nephrology and Endocrinology, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-8655, Japan.
authors.forename: Satoshi
authors.initials: S
authors.lastname: Kinugasa
authors.affiliation: Division of Nephrology and Endocrinology, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-8655, Japan.
authors.forename: Yoichiro
authors.initials: Y
authors.lastname: Ikeda
authors.affiliation: Department of Pathology, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-8655, Japan.
authors.forename: Yukako
authors.initials: Y
authors.lastname: Shintani
authors.affiliation: Department of Pathology, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-8655, Japan.
authors.forename: Masashi
authors.initials: M
authors.lastname: Fukayama
authors.affiliation: Division of Nephrology and Endocrinology, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-8655, Japan.
authors.forename: Masaomi
authors.initials: M
authors.lastname: Nangaku
mesh_terms: D005198:Fanconi Syndrome / Q000188:drug therapy / Q000209:etiology / Q000378:metabolism*; D005260:Female; D006801:Humans; D007145:Immunoglobulin kappa-Chains / Q000378:metabolism*; D007687:Kidney Tubules, Proximal / Q000378:metabolism* / Q000473:pathology; D008875:Middle Aged; D009101:Multiple Myeloma / Q000209:etiology / Q000378:metabolism; D027361:Organic Anion Transporters / Q000378:metabolism; D027701:Organic Cation Transport Proteins / Q000378:metabolism; D010018:Osteomalacia / Q000378:metabolism; D010447:Peptide Hydrolases / Q000378:metabolism; D051297:Sodium-Glucose Transporter 2 / Q000378:metabolism; D000254:Sodium-Potassium-Exchanging ATPase / Q000378:metabolism
chemical_list: D007145:Immunoglobulin kappa-Chains; D027361:Organic Anion Transporters; D027701:Organic Cation Transport Proteins; C491241:SLC22A12 protein, human; C089180:SLC5A2 protein, human; D051297:Sodium-Glucose Transporter 2; D010447:Peptide Hydrolases; D000254:Sodium-Potassium-Exchanging ATPase
keywords: Fanconi syndrome; Myeloma; NaPi-IIa; Proximal tubule; SGLT2; URAT1; κ light chain