Reactome | COL4A4 [endoplasmic reticulum lumen]

COL4A4 [endoplasmic reticulum lumen]

Stable Identifier

R-HSA-1980248

Type

Protein [EntityWithAccessionedSequence]

Species

Homo sapiens

Compartment

endoplasmic reticulum lumen

Synonyms

Collagen alpha-4(IV) chain

Locations in the PathwayBrowser

Expand all

Extracellular matrix organization (Homo sapiens)

- Collagen formation (Homo sapiens)
  - Collagen biosynthesis and modifying enzymes (Homo sapiens)
    - Collagen chain trimerization (Homo sapiens)
      - Association of procollagen type IV (Homo sapiens)
        
        Alpha-4(IV) chains [endoplasmic reticulum lumen] (Homo sapiens)
        
        COL4A4 [endoplasmic reticulum lumen] (Homo sapiens)
        
        C-linked procollagen type IV trimers [endoplasmic reticulum lumen] (Homo sapiens)
        
        C-linked procollagen alpha-3(IV), alpha-4(IV) alpha-5(IV) trimer [endoplasmic reticulum lumen] (Homo sapiens)
        
        Alpha-4(IV) chains [endoplasmic reticulum lumen] (Homo sapiens)
        
        COL4A4 [endoplasmic reticulum lumen] (Homo sapiens)
    - P4HB binds Collagen chains (Homo sapiens)
      - Collagen propeptides [endoplasmic reticulum lumen] (Homo sapiens)
        
        COL4A4 [endoplasmic reticulum lumen] (Homo sapiens)
      - P4HB:Collagen propeptides [endoplasmic reticulum lumen] (Homo sapiens)
        
        Collagen propeptides [endoplasmic reticulum lumen] (Homo sapiens)
        
        COL4A4 [endoplasmic reticulum lumen] (Homo sapiens)
    - Procollagen lysyl hydroxylases convert collagen lysines to 5-hydroxylysines (Homo sapiens)
      - Lysyl hydroxylases:Lysyl hydroxylase procollagen substrates [endoplasmic reticulum membrane] (Homo sapiens)
        
        Lysyl hydroxylase procollagen substrates [endoplasmic reticulum lumen] (Homo sapiens)
        
        Collagen propeptides, chains [endoplasmic reticulum lumen] (Homo sapiens)
        
        COL4A4 [endoplasmic reticulum lumen] (Homo sapiens)
    - Procollagen triple helix formation (Homo sapiens)
      - Collagen and procollagen triple helices:Serpin H1 [endoplasmic reticulum lumen] (Homo sapiens)
        
        Collagen and procollagen triple helices [endoplasmic reticulum lumen] (Homo sapiens)
        
        Non-fibrillar collagens [endoplasmic reticulum lumen] (Homo sapiens)
        
        Tropocollagen type IV [endoplasmic reticulum lumen] (Homo sapiens)
        
        Tropocollagen alpha-3(IV), alpha-4(IV), alpha-5(IV) [endoplasmic reticulum lumen] (Homo sapiens)
        
        Alpha-4(IV) chains [endoplasmic reticulum lumen] (Homo sapiens)
        
        COL4A4 [endoplasmic reticulum lumen] (Homo sapiens)
      - Procollagen C-linked trimers [endoplasmic reticulum lumen] (Homo sapiens)
        
        C-linked procollagen type IV trimers [endoplasmic reticulum lumen] (Homo sapiens)
        
        C-linked procollagen alpha-3(IV), alpha-4(IV) alpha-5(IV) trimer [endoplasmic reticulum lumen] (Homo sapiens)
        
        Alpha-4(IV) chains [endoplasmic reticulum lumen] (Homo sapiens)
        
        COL4A4 [endoplasmic reticulum lumen] (Homo sapiens)
    - Prolyl 4-hydroxylase converts collagen prolines to 4-hydroxyprolines (Homo sapiens)
      - P4HB:Collagen propeptides [endoplasmic reticulum lumen] (Homo sapiens)
        
        Collagen propeptides [endoplasmic reticulum lumen] (Homo sapiens)
        
        COL4A4 [endoplasmic reticulum lumen] (Homo sapiens)
    - Secretion of collagens (Homo sapiens)
      - Collagens and tropocollagens:Serpin H1 [endoplasmic reticulum lumen] (Homo sapiens)
        
        Collagens and tropocollagens [endoplasmic reticulum lumen] (Homo sapiens)
        
        Non-fibrillar collagens [endoplasmic reticulum lumen] (Homo sapiens)
        
        Tropocollagen type IV [endoplasmic reticulum lumen] (Homo sapiens)
        
        Tropocollagen alpha-3(IV), alpha-4(IV), alpha-5(IV) [endoplasmic reticulum lumen] (Homo sapiens)
        
        Alpha-4(IV) chains [endoplasmic reticulum lumen] (Homo sapiens)
        
        COL4A4 [endoplasmic reticulum lumen] (Homo sapiens)

External Reference Information

External Reference

UniProt:P53420 COL4A4

Gene Names

COL4A4

Chain

signal peptide:1-38, chain:39-1690

Reference Genes

BioGPS Gene:1286 COL4A4

COSMIC (genes):COL4A4 COL4A4

CTD Gene:1286 COL4A4

dbSNP Gene:1286 COL4A4

ENSEMBL:ENSG00000081052 COL4A4

ENSEMBL_homo_sapiens_GENE:ENSG00000081052 COL4A4

HGNC:2206 COL4A4

KEGG Gene (Homo sapiens):1286 COL4A4

Monarch:1286 COL4A4

NCBI Gene:1286 COL4A4

OMIM:120131 COL4A4

UCSC:P53420 COL4A4

Reference Transcript

Other Identifiers

0001990711

11725265_at

11725266_at

1286

16909172

214602_at

229779_at

2602119

2602120

2602121

2602122

2602123

2602125

2602131

2602132

2602134

2602138

2602141

2602142

2602143

2602144

2602145

2602146

2602150

2602151

2602152

2602153

2602154

2602155

2602156

2602157

2602158

2602159

2602160

2602161

2602163

2602164

2602165

2602166

2602169

2602170

2602171

2602172

2602173

2602174

2602175

2602176

2602177

2602178

2602179

2602180

2602181

2602182

2602184

2602185

2602186

2602187

2602188

2602191

2602192

2602193

2602194

2602198

2602199

2602200

2602201

2602202

2602209

2602212

2602216

2602217

35493_at

8059477

88649_at

A0A2R8Y548

A_23_P254522

A_33_P3227400

AAY14670

AAY24061

AB008496

AC073149

AC079235

BAA04214

BAA25065

CAA56943

CAA76763

CCDS42828

COL4A4

COL4A4-201

D17391

D17391_at

ENSG00000081052

ENSP00000379866

ENST00000396625

EntrezGene:1286

GE87916

GO:0000902

GO:0001525

GO:0001886

GO:0003674

GO:0005198

GO:0005201

GO:0005575

GO:0005576

GO:0005581

GO:0005587

GO:0005604

GO:0005615

GO:0005622

GO:0005623

GO:0005737

GO:0005783

GO:0005788

GO:0008150

GO:0030154

GO:0030198

GO:0031012

GO:0032836

GO:0032991

GO:0043226

GO:0048646

GO:0048856

HGNC:2206

Hs.119471.0.A1_3p_at

Hs.180828.0.S2_3p_at

ILMN_1778308

IPR001442

IPR008160

IPR016187

IPR036954

LRG_231

MIM:120131

MIM:203780

NM_000092

NP_000083

PF01391

PF01413

PH_hs_0023458

SM00111

TC02002832.hg

TC02004836.hg

uc061teu.1

UPI000013D987

X81053

XM_005246281

XM_005246282

XM_006712246

XM_011510557

XM_011510558

XM_011510559

XM_011510560

XM_011510561

XM_011510562

XM_011510565

XM_011510566

XM_011510567

XM_011510568

XM_011510569

XM_011510570

XM_017003296

XM_017003297

XM_017003298

XM_017003299

XM_017003300

XP_005246338

XP_005246339

XP_006712309

XP_011508859

XP_011508860

XP_011508861

XP_011508862

XP_011508863

XP_011508864

XP_011508867

XP_011508868

XP_011508869

XP_011508870

XP_011508871

XP_011508872

XP_016858785

XP_016858786

XP_016858787

XP_016858788

XP_016858789

XR_001738602

XR_001738603

XR_001738604

XR_001738605

XR_001738606

XR_001738607

XR_922837

Y17397

Y17398

Y17399

Y17400

Y17401

Y17402

Y17403

Y17404

Y17405

Y17406

Y17407

Y17408

Y17409

Y17410

Y17411

Y17412

Y17413

Y17414

Y17415

Y17416

Y17417

Y17418

Y17419

Y17420

Y17421

Y17422

Y17423

Y17424

Y17425

Y17426

Y17427

Y17428

Y17429

Y17430

Y17431

Y17432

Y17433

Y17434

Y17435

Y17436

Y17437

Y17438

Y17439

Y17440

Y17441

Y17442

Y17443

Participant Of

hasCandidate

hasMember

Alpha-4(IV) chains [endoplasmic reticulum lumen]

Other forms of this molecule

3x4Hyp-GlcGalHyl-COL4A4 [endoplasmic reticulum lumen]

GlcGalHyl-COL4A4 [endoplasmic reticulum lumen]

3x4Hyp-GalHyl-COL4A4 [endoplasmic reticulum lumen]

3x4Hyp-3Hyp-GalHyl-COL4A4 [endoplasmic reticulum lumen]

3x4Hyp-3Hyp-GlcGalHyl-COL4A4 [endoplasmic reticulum lumen]

GalHyl-COL4A4 [endoplasmic reticulum lumen]

3x4Hyp-COL4A4 [endoplasmic reticulum lumen]

3x4Hyp-3Hyp-COL4A4 [endoplasmic reticulum lumen]

3x4Hyp-5Hyl-COL4A4 [endoplasmic reticulum lumen]

3x4Hyp-3Hyp-5Hyl-COL4A4 [endoplasmic reticulum lumen]

5Hyl-COL4A4 [endoplasmic reticulum lumen]

5-sulfiliminyl collagen alpha-4(IV) chain [extracellular region]

3,4-Hyp 5-sulfiliminyl collagen alpha-4(IV) chain [extracellular region]

4-Hyp 5-sulfiliminyl collagen alpha-4(IV) chain [extracellular region]

3x4Hyp-COL4A4(39-?) [extracellular region]

3x4Hyp-3Hyp-GalHyl-COL4A4(39-?) [extracellular region]

3x4Hyp-GalHyl-COL4A4(39-?) [extracellular region]

3x4Hyp-5Hyl-COL4A4(?-1690) [extracellular region]

3x4Hyp-3Hyp-GlcGalHyl-COL4A4(?-1690) [extracellular region]

3x4Hyp-3Hyp-COL4A4(39-?) [extracellular region]

GalHyl-COL4A4(39-?) [extracellular region]

GlcGalHyl-COL4A4(?-1690) [extracellular region]

COL4A4(39-?) [extracellular region]

3x4Hyp-GalHyl-COL4A4(?-1690) [extracellular region]

3x4Hyp-3Hyp-5Hyl-COL4A4(?-1690) [extracellular region]

5Hyl-COL4A4(39-?) [extracellular region]

3x4Hyp-GalHyl-COL4A4(?-1690) [extracellular region]

COL4A4(?-1690) [extracellular region]

3x4Hyp-3Hyp-GalHyl-COL4A4(?-1690) [extracellular region]

5Hyl-COL4A4(?-1690) [extracellular region]

GlcGalHyl-COL4A4(39-?) [extracellular region]

3x4Hyp-3Hyp-GlcGalHyl-COL4A4(39-?) [extracellular region]

3x4Hyp-5Hyl-COL4A4(39-?) [extracellular region]

3x4Hyp-COL4A4(?-1690) [extracellular region]

3x4Hyp-3Hyp-5Hyl-COL4A4(39-?) [extracellular region]

3x4Hyp-GlcGalHyl-COL4A4(39-?) [extracellular region]

3x4Hyp-3Hyp-COL4A4(?-1690) [extracellular region]

GalHyl-COL4A4(?-1690) [extracellular region]

COL4A4(39-?) [extracellular region]

3x4Hyp-5Hyl-COL4A4 [extracellular region]

COL4A4 [extracellular region]

GalHyl-COL4A4 [extracellular region]

3,4-Hyp Glu-Gal-Hyl collagen alpha-4(IV) NC1 domain [extracellular region]

4-Hyp 5-Gal-Hyl collagen alpha-4(IV) NC1 domain [extracellular region]

5-Gal-Hyl collagen alpha-4(IV) NC1 domain [extracellular region]

3,4-hydroxyprolyl collagen alpha-4(IV) NC1 domain [extracellular region]

Glu-Gal-Hyl-collagen alpha-4(IV) NC1 domain [extracellular region]

5-hydroxylysyl collagen alpha-4(IV) NC1 domain [extracellular region]

3x4Hyp-3Hyp-GalHyl-COL4A4 NC1 domain [extracellular region]

4-Hyp 5-Hyl collagen alpha-4(IV) NC1 domain [extracellular region]

3,4-Hyp 5-Hyl collagen alpha-4(IV) NC1 domain [extracellular region]

4-Hyp Glu-Gal-Hyl collagen alpha-4(IV) NC1 domain [extracellular region]

4-hydroxyprolyl collagen alpha-4(IV) NC1 domain [extracellular region]

Collagen alpha-4(IV) NC1 domain [extracellular region]

3x4Hyp-GalHyl-COL4A4 [extracellular region]

3x4Hyp-3Hyp-COL4A4 [extracellular region]

3x4Hyp-3Hyp-GlcGalHyl-COL4A4 [extracellular region]

GlcGalHyl-COL4A4 [extracellular region]

3x4Hyp-GalHyl-COL4A4 [extracellular region]

5Hyl-COL4A4 [extracellular region]

3x4Hyp-COL4A4 [extracellular region]

3x4Hyp-3Hyp-GalHyl-COL4A4 [extracellular region]

3x4Hyp-3Hyp-5Hyl-COL4A4 [extracellular region]

Inferred To

Homologues of COL4A4 [endoplasmic reticulum lumen] (Canis familiaris)

Homologues of COL4A4 [endoplasmic reticulum lumen] (Sus scrofa)

COL4A4 [endoplasmic reticulum lumen] (Bos taurus)

COL4A4 [endoplasmic reticulum lumen] (Canis familiaris)

COL4A4 [endoplasmic reticulum lumen] (Mus musculus)

COL4A4 [endoplasmic reticulum lumen] (Sus scrofa)

COL4A4 [endoplasmic reticulum lumen] (Taeniopygia guttata)

Cross References

RefSeq

XP_005246338.1, NP_000083.3

OpenTargets

ENSG00000081052

GeneCards

P53420

PRO

P53420

Orphanet

15773

PDB

5NB1