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. 2015 Feb 5;96(2):318–328. doi: 10.1016/j.ajhg.2014.12.022

Table 2.

Gene Expression Pathways Significantly Associated with Consortium Lung Phenotype

Pathway
Genes
Statistics

ID Name Number a b Trendc p Valued q Valuee Genes with Gene-Level p Value < 0.05 (Ordered by p Value)f
GO Cellular Component Pathways

0001673 male germ cell nucleus 14 0 14 down 0.0001 0.0164 TNP1; REC8; TCFL5
0012507 ER to Golgi transport vesicle membrane 25 23 2 up 0.0003 0.0481 HLA-E; MCFD2; TMED7; HLA-F
0043073 germ cell nucleus 17 1 16 down 0.0004 0.0442 TNP1; REC8; TCFL5
0042470;0048770 melanosome; pigment granule 78 52 26 up 0.0007 0.0582 SLC3A2; TPP1; CTSD; ANXA2; STOM; HSPA5; BSG
0030134 ER to Golgi transport vesicle 29 25 4 up 0.0011 0.0737 HLA-E; MCFD2; TMED7; HLA-F
0030176 integral to endoplasmic reticulum membrane 85 64 21 up 0.0024 0.1181 TTC35; HLA-E; EDEM1; TAP1; SELS; HLA-F; HSPA5; MMGT1
0031301 integral to organelle membrane 171 113 58 up 0.0026 0.1181 TTC35; HLA-E; EDEM1; TAP1; SELS; ST6GALNAC6; HLA-F; A4GALT; ARMCX3; P2RX7; LARGE; HSPA5; MMGT1
0000421 autophagic vacuole membrane 13 11 2 up 0.0028 0.1181 WIPI1; ATG9A
0031227 intrinsic to endoplasmic reticulum membrane 95 70 25 up 0.0031 0.1181 TTC35; HLA-E; EDEM1; TAP1; SELS; HLA-F; HSPA5; MMGT1
0031300 intrinsic to organelle membrane 184 121 63 up 0.0036 0.1231 TTC35; HLA-E; EDEM1; TAP1; SELS; ST6GALNAC6; HLA-F; A4GALT; ARMCX3; P2RX7; LARGE; HSPA5; MMGT1
0030658 transport vesicle membrane 49 33 16 up 0.0039 0.1231 HLA-E; MCFD2; TMED7; HLA-F; NCALD

GO Biological Process Pathways

0006518 peptide metabolic process 64 46 18 up 0.0001 0.0837 GSTK1; DNPEP; PSEN2; TPP1
0072384 organelle transport along microtubule 24 21 3 up 0.0001 0.0837 PRKCZ; COPG
0006925 inflammatory cell apoptotic process 10 10 0 up 0.0003 0.1107 none
0006944 cellular membrane fusion 61 42 19 up 0.0003 0.1107 CD9; PLDN; ANXA2; BET1
0007030 golgi organization 38 28 10 up 0.0003 0.1107 GCC2; BHLHA15; GOLGB1; PLK3; COG1; TMED2
0043603 cellular amide metabolic process 101 65 36 up 0.0003 0.1107 GSTK1; DNPEP; PSEN2; TPP1; PRKCD
0034067 protein localization to Golgi apparatus 14 13 1 up 0.0004 0.1166 GOLGA4; GCC2; ATG9A
0045684 positive regulation of epidermis development 11 10 1 up 0.0004 0.1166 none

GO Molecular Function Pathways

0050839 cell adhesion molecule binding 33 15 18 two sided 0.0004 0.1181 P2RX4; MLLT4; CD1D;gCTNNA1; PVRL1g
0042287 MHC protein binding 15 9 6 two sided 0.0006 0.1191 TAP1; LAG3; MARCH8

MSigDB Pathways

ATAAGCT.MIR.21 81 45 36 two sided 0.0001 0.0387 BAHD1; BTBD3;gC5orf41; STK40; UBR3; NF2;gSSFA2; JAG1; PPARA; PELI1; RHOB; CREBL2
V.HMGIY_Q6 158 70 88 two sided 0.0006 0.1499 ZNF675;gLMO4; TNFSF11;gPLAGL2; POLD3;gSLC7A1; UBE2E2;gTAZ; UBR3; MRC2;gTNFSF4; IKZF2g

MetaMiner Cystic Fibrosis Specific Pathwaysh

cholesterol and sphingolipids transport/recycling to plasma membrane in lung (normal and CF) 14 9 5 two sided 0.0036 0.0597 ABCG1g
normal wtCFTR traffic/sorting endosome formation 14 11 3 up 0.0052 0.0621 none
F508-CFTR traffic/ER-to-Golgi in CF; Normal wtCFTR traffic/ER-to-Golgi 22 20 2 up 0.0075 0.0621 COPG; COPZ2
mucin expression in CF via TLRs, EGFR signaling pathways 48 34 14 up 0.0116 0.0770 JUN; PRKCD

PFAM Pathways

00035 double-stranded RNA binding motif 17 2 15 down 0.0001 0.0135 STRBP; STAU2
07716 basic region leucine zipper 11 7 4 two sided 0.0002 0.0276 DDIT3; CREBL2; CEBPB
03953 tubulin C-terminal domain 15 2 13 down 0.0009 0.0804 TUBB2B

CF Relevant Custom Pathways

ER stress response 169 127 42 up 0.0005 0.0106 DNAJB9; EDEM1; CISD2; TANK; DDIT3; SERP1; FDPS; LONP1; NANS; SSR4; JUN; GADD45A; LY9; PGM3; HSPA5; ARF4; IER3IP1; BTG2; CEBPB; CNIH; MANF; PDIA6
XBP1 target genes 13 10 3 two sided 0.0079 0.1165 DNAJB9; EDEM1; SERP1; PDIA6

HLA-Specific Pathways

class I 3 3 0 up 0.0221 0.0261 HLA-E; HLA-F
class II 8 7 1 up 0.0868 0.0580 none
class I and class II 11 10 1 up 0.0299 0.0261 HLA-E; HLA-F

Pathways limited to those with ≥10 but ≤200 genes. SAFE analysis utilized 10,000 permutations to establish significance thresholds. CF Relevant Custom Pathways developed primarily as described for mice46 using human gene counterparts (Table S8).

a

Number of genes in pathway with increased expression.

b

Number of genes in pathway with decreased expression.

c

Up (increased) or down (decreased) differential expression of genes in the pathways associated with milder lung disease. Two-sided indicates pathways that contained both increased and decreased differentially expressed genes that contributed significantly to the signal.

d

Determined by 10,000 permutations in the SAFE package.22

e

Benjamini-Hochberg false-discovery for pathways testing within each pathway set; q values < 0.15 were included.

f

See Table S6 (tab A) for the inclusive list of genes for these pathways; “none” indicates that no individual genes within the pathway had a p value less than 0.05; see Table S5 for gene MIM numbers.

g

For the two-sided “Trend,” these genes have a “down” trend.

h

MetaMiner CF Specific Pathways represent a version of Thomson Reuters’ (formerly GeneGo) MetaDiscovery suite that is enriched with content specific for cystic fibrosis.