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Pathways of neurodegeneration - multiple diseases

KEGG ID: hsa05022

中文名称

肺表面活性物质代谢

通路描述

肺泡区域形成广泛的上皮表面,介导出生后呼吸所需的氧气和二氧化碳转移。I 型上皮细胞形成肺泡表面并介导气体交换。II 型上皮细胞分泌肺表面活性物质,这是一种脂蛋白复合物,形成薄界面膜,降低肺泡气液界面的表面张力,维持肺泡的结构完整性,防止其在低体积下塌陷(Agassandian & Mallampalli 2013)。出生前表面活性物质产生增加,以准备出生时呼吸空气(Hallman 2013)。早产儿由于 II 型上皮细胞尚未完全分化,产生的表面活性物质不足,可能导致呼吸窘迫综合征。表面活性物质主要由富含磷脂的磷脂酰胆碱(PC)和磷脂酰甘油(PG)组成(Agassandian & Mallampalli 2013),以及肺表面活性蛋白 A、B、C 和 D(SFTPA-D)。它们调节表面活性物质的稳态,有助于脂质在肺泡中的物理结构,并调节表面活性物质的功能和代谢。SFTPA 和 D 是大分子亲水性蛋白,而 SFTPB 和 C 是小分子非常疏水性蛋白(Johansson et al. 1994)。除了表面活性物质功能外,SFTPA 和 D 在先天宿主防御中发挥重要作用,通过结合并清除入侵的微生物从肺部(Kingma & Whitsett 2006)。此处描述了表面活性物质的核调节、运输、代谢、再利用和降解(Ikegami 2006, Boggaram 2009, Whitsett et al. 2010)。参与这些过程的基因突变可导致呼吸窘迫综合征、肺蛋白症、间质性肺疾病和慢性肺疾病(Perez-Gil & Weaver 2010, Whitsett et al. 2010, Akella & Deshpande 2013, Jo 2014)。
英文描述
Neurodegeneration is generally defined as progressive, irreversible loss of neurons, which may affect either the peripheral or central nervous system (CNS). Neurodegenerative diseases (NDs) include highly debilitating illnesses, such as Alzheimer (AD), Parkinson disease (PD), amyotrophic lateral sclerosis, Huntington disease, spinocerebellar ataxias, and prion diseases (PrD). The hallmark event, which is thought to be at the root of these diseases, is the progressive accumulation of misfolded protein aggregates. Major basic processes include abnormal protein dynamics due to deficiency of the ubiquitin-proteosome-autophagy system, oxidative stress and free radical formation, endoplasmic reticulum stress, mitochondrial dysfunction and (secondary) disruptions of axonal transport.

所含基因

479 个基因
ACTR10 ACTR1A ACTR1B ADRM1 AGER ALS2 AMBRA1 APAF1 APC APC2 APP ARAF ATF4 ATF6 ATG101 ATG13 ATG14 ATG2A ATG2B ATP2A1 ATP2A2 ATP2A3 ATP5F1A ATP5F1B ATP5F1C ATP5F1D ATP5F1E ATP5MC1 ATP5MC2 ATP5MC3 ATP5PB ATP5PD ATP5PF ATP5PO ATP6 ATP8 ATXN1 ATXN1L ATXN2 ATXN2L ATXN3 ATXN3L AXIN1 AXIN2 BAD BAK1 BAX BCL2 BCL2L1 BDNF BECN1 BECN2 BID BRAF CACNA1B CACNA1C CACNA1D CACNA1F CACNA1S CALM1 CALM2 CALM3 CALML3 CALML4 CALML5 CALML6 CAMK2A CAMK2B CAMK2D CAMK2G CAPN1 CAPN2 CASP12 CASP3 CASP7 CASP8 CASP9 CAT CCS CDK5 CDK5R1 CHMP2B CHRFAM7A CHRM1 CHRM3 CHRM5 CHRNA7 COX1 COX2 COX3 COX4I1 COX4I2 COX5A COX5B COX6A1 COX6A2 COX6B1 COX6B2 COX6C COX7A1 COX7A2 COX7A2L COX7B COX7B2 COX7C COX8A COX8C COXFA4 COXFA4L2 CSF1 CSNK1A1 CSNK1A1L CSNK1E CSNK2A1 CSNK2A2 CSNK2A3 CSNK2B CTNNB1 CYBB CYC1 CYCS CYTB DAXX DCTN1 DCTN2 DCTN3 DCTN4 DCTN5 DCTN6 DDIT3 DERL1 DKK1 DKK2 DKK4 DLG4 DNAH1 DNAH10 DNAH11 DNAH12 DNAH14 DNAH17 DNAH2 DNAH3 DNAH5 DNAH6 DNAH7 DNAH8 DNAH9 DNAI1 DNAI2 DNAL1 DNAL4 DNALI1 DVL1 DVL2 DVL3 EIF2AK3 EIF2S1 ERN1 FADD FAS FASLG FIG4 FRAT1 FRAT2 FUS FZD1 FZD10 FZD2 FZD3 FZD4 FZD5 FZD6 FZD7 FZD8 FZD9 GNAQ GPR37 GPX1 GPX2 GPX3 GPX5 GPX6 GPX7 GPX8 GRIA1 GRIA2 GRIA3 GRIA4 GRIN1 GRIN2A GRIN2B GRIN2C GRIN2D GRM1 GRM5 GSK3B HAP1 HIP1 HRAS HSD17B10 HSPA5 HTRA2 HTT IFT57 IL1A IL1B IL6 ITPR1 ITPR2 ITPR3 KIF5A KIF5B KIF5C KLC1 KLC2 KLC3 KLC4 KRAS LOC107984365 LRP5 LRP6 LRRK2 MAP1LC3A MAP1LC3B MAP1LC3B2 MAP1LC3BP1 MAP1LC3C MAP2K1 MAP2K2 MAP2K3 MAP2K6 MAP2K7 MAP3K10 MAP3K5 MAPK1 MAPK10 MAPK11 MAPK12 MAPK13 MAPK14 MAPK3 MAPK8 MAPK9 MAPT MCU MFN1 MFN2 MTOR ND1 ND2 ND3 ND4 ND4L ND5 ND6 NDUFA1 NDUFA10 NDUFA11 NDUFA12 NDUFA13 NDUFA2 NDUFA3 NDUFA5 NDUFA6 NDUFA7 NDUFA8 NDUFA9 NDUFAB1 NDUFB1 NDUFB10 NDUFB11 NDUFB2 NDUFB3 NDUFB4 NDUFB5 NDUFB6 NDUFB7 NDUFB8 NDUFB9 NDUFC1 NDUFC2 NDUFC2-KCTD14 NDUFS1 NDUFS2 NDUFS3 NDUFS4 NDUFS5 NDUFS6 NDUFS7 NDUFS8 NDUFV1 NDUFV2 NDUFV3 NEFH NEFL NEFM NFKB1 NOS1 NOS2 NOX1 NOX4 NRAS NRBF2 OPTN PARK7 PDYN PIK3C3 PIK3R4 PINK1 PLCB1 PLCB2 PLCB3 PLCB4 PLCG1 PPID PPIF PPP3CA PPP3CB PPP3CC PPP3R1 PPP3R2 PRKCA PRKCB PRKCG PRKN PRNP PRPH PSEN1 PSEN2 PSMA1 PSMA2 PSMA3 PSMA4 PSMA5 PSMA6 PSMA7 PSMA8 PSMB1 PSMB2 PSMB3 PSMB4 PSMB5 PSMB6 PSMB7 PSMC1 PSMC2 PSMC3 PSMC4 PSMC5 PSMC6 PSMD1 PSMD11 PSMD12 PSMD13 PSMD14 PSMD2 PSMD3 PSMD4 PSMD6 PSMD7 PSMD8 PSMD9 PTGS2 RAB1A RAB39B RAB5A RAB8A RAC1 RAF1 RB1CC1 RELA RPS27A RYR1 RYR2 RYR3 SDHA SDHB SDHC SDHD SEM1 SEPTIN5 SIGMAR1 SLC25A31 SLC25A4 SLC25A5 SLC25A6 SLC6A3 SMCR8 SNCA SNCAIP SOD1 SPG11 SPTBN2 SQSTM1 STX1A TANK TARDBP TBK1 TNF TNFRSF1A TNFRSF1B TOMM40 TOMM40L TPTEP2-CSNK1E TRAF2 TRAP1 TRPC3 TUBA1A TUBA1B TUBA1C TUBA3C TUBA3D TUBA3E TUBA4A TUBA8 TUBAL3 TUBB TUBB1 TUBB2A TUBB2B TUBB3 TUBB4A TUBB4B TUBB6 TUBB8 TUBB8B UBA1 UBA52 UBA7 UBB UBC UBE2G1 UBE2G2 UBE2J1 UBE2J2 UBE2L3 UBE2L6 UCHL1 ULK1 ULK2 UQCR10 UQCR11 UQCRB UQCRC1 UQCRC2 UQCRFS1 UQCRH UQCRHL UQCRQ VAPB VCP VDAC1 VDAC2 VDAC3 WDR41 WIPI1 WIPI2 WNT1 WNT10A WNT10B WNT11 WNT16 WNT2 WNT2B WNT3 WNT3A WNT4 WNT5A WNT5B WNT6 WNT7A WNT7B WNT8A WNT8B WNT9A WNT9B XBP1 ZFYVE1