おすすめの製品
アッセイ
≥98% (HPLC)
形状
powder
色
white to beige
溶解性
DMSO: 2 mg/mL, clear
保管温度
2-8°C
生物化学的／生理学的作用
QX77 is a chaperone-mediated autophagy (CMA) activator derived from the atypical retinoid AR7 that activates CMA by antagonizing retinoic acid receptor-α (RARα) signaling. QX77 rescues defective trafficking & lysosomal localization of the CMA receptor LAMP2A in cystinotic Ctns-/- MEFs and CTNS-KO human proximal tubule cells (PTCs) by restoring Rab11 expression and Rab11-positive vesicles trafficking to the level seen in wild-type cells (20 μM, 48 hrs). CMA activation by QX77 treatment (20 μM, 5 days) also significantly reduces toxic α-synuclein (α-syn) accumulation in PD patients iPSCs-derived astrocytes with LRRK2 G2019S mutation.
保管分類コード
13 - Non Combustible Solids
WGK
WGK 3
引火点(°F)
Not applicable
引火点(℃)
Not applicable
適用法令
試験研究用途を考慮した関連法令を主に挙げております。化学物質以外については、一部の情報のみ提供しています。 製品を安全かつ合法的に使用することは、使用者の義務です。最新情報により修正される場合があります。WEBの反映には時間を要することがあるため、適宜SDSをご参照ください。
Jan Code
SML2634-5MG:
SML2634-VAR:
SML2634-BULK:
SML2634-25MG:
試験成績書（COA）
製品のロット番号・バッチ番号を入力して、試験成績書（COA） を検索できます。ロット番号・バッチ番号は、製品ラベルに「Lot」または「Batch」に続いて記載されています。
Jaime Anguiano et al.
Nature chemical biology, 9(6), 374-382 (2013-04-16)
Chaperone-mediated autophagy (CMA) contributes to cellular quality control and the cellular response to stress through the selective degradation of cytosolic proteins in lysosomes. A decrease in CMA activity occurs in aging and in age-related disorders (for example, neurodegenerative diseases and
Angelique di Domenico et al.
Stem cell reports, 12(2), 213-229 (2019-01-15)
Parkinson's disease (PD) is associated with the degeneration of ventral midbrain dopaminergic neurons (vmDAns) and the accumulation of toxic α-synuclein. A non-cell-autonomous contribution, in particular of astrocytes, during PD pathogenesis has been suggested by observational studies, but remains to be
Jinzhong Zhang et al.
Frontiers in endocrinology, 10, 21-21 (2019-02-19)
Cystinosis is a lysosomal storage disorder caused by defects in CTNS, the gene that encodes the lysosomal cystine transporter cystinosin. Patients with nephropathic cystinosis are characterized by endocrine defects, defective proximal tubule cell (PTC) function, the development of Fanconi syndrome
Jinzhong Zhang et al.
The Journal of biological chemistry, 292(25), 10328-10346 (2017-05-04)
The lysosomal storage disease cystinosis, caused by cystinosin deficiency, is characterized by cell malfunction, tissue failure, and progressive renal injury despite cystine-depletion therapies. Cystinosis is associated with defects in chaperone-mediated autophagy (CMA), but the molecular mechanisms are incompletely understood. Here
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