TABLE 2.
List of peroxisomal proteins involved in α- and β-oxidation in humans, chromosome mapping, tissue expression, and targeting
| Approved Gene Symbol | Approved Gene Name | Aliases | Accession No. | Locus | Tissue Expression | Targeting Sequence | Remarksb |
|---|---|---|---|---|---|---|---|
| ABCD1 | ATP-binding cassette, subfamily D (ALD), member 1 | AMN, ALDP, ALD, ABC42 | NM_000033 | Xq28 | G (high in cervix, pancreas, skin; nerves; low in liver) | mPTS | |
| ABCD2 | ATP-binding cassette, subfamily D (ALD), member 2 | ALDL1, ALDR, ABC39, ALDL1, ALDRP, hALDR | NM_005164.3 | 12q11-q12 | R (brain, liver) | mPTS | Low expression compared with ABCD1 |
| ABCD3 | ATP-binding cassette, subfamily D (ALD), member 3 | PMP70, ABC43; PXMP1 | NM_002858 | 1p22-p21 | U | mPTS | Not expressed in adipose |
| ACAA1 | Acetyl-CoA acyltransferase 1 | Peroxisomal thiolase, THIOL | NM_001607 | 3p22-p21.3 | U (high in liver, spleen; intestines) | PTS2 | Likely splice forms |
| ACAD11 | Acyl-CoA dehydrogenase family, member 11 | FLJ12592; MGC150619 | NM_032169 | 3q22.1 | U (high in kidney, thymus) | TAKI | |
| ACNAT1 | Acyl-CoA amino acid N-acyltransferase | AL359893 | 9q22.3 | RSKL | Pseudogene of BAAT | ||
| ACSL1 | Acyl-CoA synthetase long-chain family member 1 | LACS2, LACS, ACS1, LACS1, FACL1 | NM_001995 | 4q35 | U (high in adipose tissue, mammary gland, adrenals, liver) | TIKV | Active on C16:0, Phyt, Pris |
| ACSL4 | Acyl-CoA synthetase long-chain family member 4 | FACL4, ACS4, LACS4 | NM_004458 | Xq22.3-q23 | U | Membrane associated ? | Peripheral membrane protein in rat |
| SLC27A2 | Solute carrier family 27 (FA transporter), member 2 | ACSVL1FATP2, hFACVL1, VLACS, VLCS | NM_003645 | 15q21.2 | G (high in kidney, liver, placenta) | TLKL ? | Also ER associated active on Phyt, Pris, C24:0, THCA |
| SLC27A4 | Solute carrier family 27 (FA transporter), member 4 | ACSVL5FATP4 | NM_005094.2 | 9q34.13 | G (high in skin) | EEKL ?(peripheral) | Also mitochondria, MAM, ER in fibroblasts |
| ACOX1 | Acyl-CoA oxidase 1a acyl-CoA oxidase 1 b | ACOX, SCOX, PALMCOX, palmitoyl-CoA oxidase | NM_004035 NM_007292 | 17q25.1 | G | QSKL QSKL | Two splice forms with different exon 3 |
| ACOX2 | Acyl-CoA oxidase 2 | BCOX, BRCOX, THCCox, BRCACOX, branched chain acyl-CoA oxidase | NM_003500 | 3p14.3 | R (liver, nerves) | RSKL | |
| ACOX3 | Acyl-CoA oxidase 3 | Pristanoyl-CoA oxidase | NM_003501 | 4p15.3 | G (prostate, adrenals) | KSKL | Present as protein ? a shorter C-truncated splice form exists (NM_001101667) |
| ACOXL | Acyl-CoA oxidase-like | ACOX4 | NM_018308 | 2q13 | R (lung) | absent | Major transcript truncated; a splice form ending in GAKL might be targeted |
| ACOT2 | Acyl-CoA thioesterase 2 | Mte1, PTE2 | NM_006821 | 14q24.3 | G (liver>kidney) | PSKV | Likely only mitochondrial; active on medium and long-chain esters |
| ACOT4 | Acyl-CoA thioesterase 4 | PTE-Ib; PTE1B; PTE2B | NM_152331 | 14q24.3 | R (liver<kidney; present in adipose tissue) | VPKL | Combined activities of mouse ACOT3,4,5; active on succinyl-, medium, and long-chain CoA-esters |
| ACOT6 | Acyl-CoA thioesterase 6 | Pristanoyl-phytanoyl-CoA thioesterase | NM_001037162 | 14q24.3 | R (testis, connective tissue) | HSKI ? | Compared with mouse counterpart, lacking first exon with startcodon; not targeted in fibroblasts |
| ACOT8 | Acyl-CoA thioesterase 8 | hACTE-III, PTE-2, PTE1, TE2, het, HNAACTE | NM_005469.2 | 20q12-q13.1 | U | ESKL | Active on diC5 to diC12-, choloyl-, prostaglandin-, pristanoyl-CoA; interacts with NEF |
| ACOT12 | Acyl-CoA thioesterase 12 | Cach, THEAL, STARD15 | NM_130767 | 5q14.1 | R (liver specific) | VSTF ?? | |
| ALDH3A2 | Aldehyde dehydrogenase 3 family, member A2 | SLS, ALDH10, FALDH | NM_000382.2 | 17p11.2 | U | SKQR ? | Major splice variant FALDH-V (membrane bound); ER-localized isoform FALDH-N corresponds to NM_001031806.1 |
| AMACR | α-Methylacyl-CoA racemase | Epimerase | NM_014324 | 5p13.2-q11.1 | U | KASLa | Different splice forms |
| BAAT | Bile acid CoA-amino acid N-acyltransferase | BAT | NM_001701.3 | 9q22.3 | R (liver specific) | TSQL | Might be partially cytoplasmic |
| CAT | Catalase | NM_001752 | 11p13 | U | KANL | ||
| CRAT | Carnitine acetyltransferase | CAT1 | NM_000755.3 | 9q34.1 | G (skeletal muscle> heart, liver and pancreas> brain, placenta, lung, kidney). | RAKL | By alternatively splicing, an isoform with an N-terminal mitochondrial transit peptide |
| CROT | Carnitine O-octanoyltransferase | COT | NM_021151 | 7q21.1 | G (low in heart, adipose tissue) | STHL | Malonyl-CoA inhibitory |
| DECR2 | 2,4-Dienoyl CoA reductase 2, peroxisomal | PDCR, SDR17C1 | NM_020664 | 16p13.3 | G/R | SAKL | |
| DHRS4 | Dehydrogenase/reductase (SDR family) member 4 | SCAD-SRL, SDR-SRL, SDR25C1 | NM_021004 | 14q11.2 | U/G | PSRL | Reduction of 3-oxosteroids; oxidation of 3β-hydroxysteroids; lower activity on retinal/retinol than pig or mouse DHRS4 |
| ECH1 | Enoyl CoA hydratase 1, peroxisomal | Δ3,5-Δ,4-dienoyl-CoA isomerase, HPXEL | NM_001398.2 | 19q13.1 | U (high in adipose tissue, muscle, heart) | FSKLa | |
| FAR1 | Fatty acyl CoA reductase 1 | SDR10E1 | NM_032228 | 11p15.2 | G | TMRY ? | Partial colocalization |
| FAR2 | Fatty acyl CoA reductase 2 | SDR10E2 | NM_018099 | 12p11.23 | R (sebaceous glands) | TLKV ? | Partial colocalization |
| HACL1 | 2-Hydroxyacyl-CoA lyase 1 | 2-HPCL | NM_012260 | 3p24.3 | U | RSNM | |
| HAO2 | Hydroxyacid oxidase 2 (long-chain) | HAOX2, GIG16 | NM_001005783 | 1p13.3-p13.1 | R (liver, kidney) | FSRL | |
| HSDL2 | Hydroxysteroid dehydrogenase like 2 | SDR13C1 | NM_032303 | 9q32 | U (kidney, liver, testis, ovary, pancreas) | NARL | C-terminal SCP2 domain; likely targeted to mitochondria as well; substrate? |
| IDH1 | Isocitrate dehydrogenase 1 (NADP+), soluble | IDH, IDP, IDCD, IDPC, PICD, IDH1 | NM_005896.2 | 2q33.3 | U | GAKL | Mainly cytosolic |
| EHHADH | Enoyl-CoA, hydratase/3-hydroxyacyl-CoA dehydrogenase | MFP1 MFE-1, L-BP, ECHD | NM_001966 | 3q26.3-q28 | G(high in liver>kidney) | SSKL | Evidence for a N-truncated isoform (NM_001166415) |
| 17HSD4 | Hydroxysteroid 17β-dehydrogenase 4 | MFP2 MFE-2, DBP, SDR8C1 | NM_000414 | 5q21 | U (high in liver, adipose, spleen, brain) | YAKL | |
| NUDT7 | Nudix (nucleoside diphosphate linked moiety X)-type motif 7 | NM_001 105663.1 | 16q23.1 | R(spleen, prostate) | TSRL | Active on acyl-CoA, CoA | |
| NUDT12 | Nudix (nucleoside diphosphate linked moiety X)-type motif 12 | NM_031438 | 5q21.2 | G (high in liver, kidney) | NPNL | Active on NAD(P)H | |
| PECI | Peroxisomal Δ3,Δ2-enoyl-CoA isomerase | ACBD2, DRS1, HCA88 | NM_006117 | 6p24.3 | U (high in liver, low brain, muscle) | KSKL | |
| PECR | Peroxisomal trans-2-enoyl-CoA reductase | TERP, SDR29C1 | NM_018441 | 2q35 | G(high in liver, kidney) | KAKL | |
| PHYH | Phytanoyl-CoA 2-hydroxylase | PAHX, PHYH1 | NM_006214 | 10p13 | U | PTS2 | |
| PXMP2 | PMP2, 22 kDa | PMP22 | NM_018663.1 | 12q24.33 | G | mPTS | |
| SCP2 | SCPx | SCPx | NM_002979 | 1p32 | U | NAKL | Evidence for C-truncated splice form (NM_001007098.1) |
| SCP2 | SCP2 | SCP2; NLTP; NSL-TP | NM_002979 | 1p32 | U | NAKL | Three variants |
| SLC25A17 | Solute carrier family 25 (mitochondrial carrier; PMP, 34 kDa), member 17 | PMP34 | NM_006358 | 22q13.2 | U (high in kidney, liver) | mPTS | |
| SLC22A21 | Solute carrier family 25 member 21 | OCNT3 | ?? | mPTS | Mouse specific ? | ||
| TYSND1 | Trypsin domain containing 1 | NM_173555.2 | 10q22.1 | R (bone marrow>lung>liver) | RSKL | Evidence for a truncated spliceform (NM_001040273.1) |
Entries are arranged alphabetically according to their gene symbol (bold) or the preferred abbreviation used in the main text (alias or name in bold). Expression, based on published tissue distribution, mRNA analysis, and/or in silico expression data (http://www.ncbi.nlm.nih.gov/unigene), is defined as ubiquitous (U, in all tissues), general (G, in most tissues), or restricted (R). The targeting sequence is defined as mPTS (for integral membrane proteins), PTS2, or PTS1. For the latter, the last four amino acids are given, because weaker tripeptides are often preceeded by a positive charged amino acid (printed bold). If the peptide sequence if followed by a question mark, the PTS1 functionality is doubtful.
a
Same protein is targeted to mitochondria via N-terminal mitochondrial sequence.
b
NEF, negative factor of immunodeficiency virus; Phyt, phytanic acid; Pris, pristanic acid.