ASZ1

Summary

ASZ1 (ankyrin repeat, SAM and basic leucine zipper domain containing 1, HGNC:1350) is a protein-coding gene on chromosome 7q31.2, encoding Ankyrin repeat, SAM and basic leucine zipper domain-containing protein 1 (Q8WWH4). Plays a central role during spermatogenesis by repressing transposable elements and preventing their mobilization, which is essential for the germline integrity.

Predicted to be involved in male meiotic nuclear division; spermatogenesis; and transposable element silencing. Predicted to be located in cytoplasm. Predicted to be active in pi-body.

Source: NCBI Gene 136991 — RefSeq curated summary.

At a glance

• Gene–disease (curated): male infertility with azoospermia or oligozoospermia due to single gene mutation (Moderate, GenCC)
• GWAS associations: 9
• Clinical variants (ClinVar): 67 total — 2 pathogenic
• MANE Select transcript: NM_130768

Identifiers

Gene identifiers

Field	Value
HGNC ID	HGNC:1350
Approved symbol	ASZ1
Name	ankyrin repeat, SAM and basic leucine zipper domain containing 1
Location	7q31.2
Locus type	gene with protein product
Status	Approved
Aliases	Orf3, GASZ, ALP1, CT1.19
Ensembl gene	ENSG00000154438
Ensembl biotype	protein_coding
OMIM	605797
Entrez	136991

Gene structure

Transcript identifiers

Ensembl transcripts: 6 — 3 retained_intron, 2 protein_coding, 1 nonsense_mediated_decay

ENST00000284629, ENST00000428663, ENST00000450714, ENST00000463182, ENST00000465832, ENST00000479454

RefSeq mRNA: 3 — MANE Select: NM_130768 NM_001301821, NM_001301822, NM_130768

CCDS: CCDS5772

Canonical transcript exons

ENST00000284629 — 13 exons

Exon	Start	End
ENSE00001129887	117363222	117363748
ENSE00001646958	117427356	117427493
ENSE00003459051	117379938	117380047
ENSE00003461790	117382986	117383110
ENSE00003478967	117426836	117426935
ENSE00003534625	117381011	117381067
ENSE00003546210	117384726	117384860
ENSE00003549886	117368612	117368717
ENSE00003557493	117367352	117367465
ENSE00003564423	117420163	117420274
ENSE00003574202	117385698	117385809
ENSE00003594457	117382069	117382144
ENSE00003627105	117422237	117422359

Expression profiles

Bgee: expression breadth broad, 60 present calls, max score 86.57.

FANTOM5 (CAGE): breadth tissue_specific, TPM avg 0.0078 / max 8.1195, expressed in 3 samples.

FANTOM5 promoters (1 alternative TSS)

Promoter ID	TPM avg	Samples expressed
85842	0.0078	3

Top tissues by expression

225 total, by Bgee expression score (0-100, higher = more expressed):

Tissue	Anatomy ID	Expression score	Quality
male germ line stem cell (sensu Vertebrata) in testis	CL:0000089 ∩ UBERON:0000473	86.57	gold quality
primordial germ cell in gonad	CL:0000670 ∩ UBERON:0000991	81.37	gold quality
right testis	UBERON:0004534	81.14	gold quality
left testis	UBERON:0004533	80.01	gold quality
testis	UBERON:0000473	79.77	gold quality
buccal mucosa cell	CL:0002336	67.85	silver quality
adult organism	UBERON:0007023	55.63	gold quality
corpus callosum	UBERON:0002336	55.38	gold quality
sperm	CL:0000019	52.25	gold quality
stromal cell of endometrium	CL:0002255	52.15	silver quality
lower lobe of lung	UBERON:0008949	50.64	silver quality
granulocyte	CL:0000094	46.39	silver quality
skeletal muscle tissue of rectus abdominis	UBERON:0004511	43.37	gold quality
upper leg skin	UBERON:0004262	42.91	gold quality
secondary oocyte	CL:0000655	42.57	gold quality
blood	UBERON:0000178	41.77	silver quality
lung	UBERON:0002048	41.72	gold quality
vastus lateralis	UBERON:0001379	41.41	gold quality
quadriceps femoris	UBERON:0001377	41.37	gold quality
superficial temporal artery	UBERON:0001614	41.33	gold quality
palpebral conjunctiva	UBERON:0001812	41.10	gold quality
mucosa of paranasal sinus	UBERON:0005030	40.98	gold quality
bone marrow	UBERON:0002371	40.88	gold quality
cortical plate	UBERON:0005343	40.79	gold quality
monocyte	CL:0000576	40.77	gold quality
amniotic fluid	UBERON:0000173	40.69	gold quality
jejunal mucosa	UBERON:0000399	40.59	gold quality
biceps brachii	UBERON:0001507	40.57	gold quality
epithelium of nasopharynx	UBERON:0001951	40.45	gold quality
myocardium	UBERON:0002349	40.45	gold quality

Single-cell (SCXA)

Detected in 1 experiment(s), a significant marker in 1.

Experiment	Marker?	Max mean expression
E-ANND-3	yes	3.91

Regulation

Is transcription factor: no

miRNA regulators (miRDB)

28 targeting ASZ1, top 30 by miRDB confidence (max_score; target_count = how many genes the miRNA targets in total — lower means more specific):

miRNA	Max score	Avg score	miRNA target_count
HSA-MIR-432-3P	100.00	67.86	705
HSA-MIR-5011-5P	100.00	83.46	5820
HSA-MIR-1193	100.00	65.93	529
HSA-MIR-4803	99.98	71.99	3117
HSA-MIR-4775	99.98	75.00	6394
HSA-MIR-302C-5P	99.97	72.56	3642
HSA-MIR-548AT-5P	99.96	70.83	2666
HSA-MIR-5688	99.96	73.23	4504
HSA-MIR-495-3P	99.96	72.81	4197
HSA-MIR-7-1-3P	99.91	71.53	4384
HSA-MIR-7-2-3P	99.91	71.40	4394
HSA-MIR-548E-5P	99.89	72.73	4486
HSA-MIR-95-5P	99.89	72.17	3973
HSA-MIR-182-5P	99.87	74.03	2589
HSA-MIR-6715A-3P	99.83	68.05	1473
HSA-MIR-4420	99.82	70.08	1624
HSA-MIR-181B-2-3P	99.81	70.06	1646
HSA-MIR-181B-3P	99.81	70.06	1646
HSA-MIR-202-5P	99.78	67.65	991
HSA-MIR-410-3P	99.27	69.98	2457
HSA-MIR-7158-5P	99.25	67.95	796
HSA-MIR-3973	99.20	69.19	1990
HSA-MIR-8070	99.07	69.30	1303
HSA-MIR-6715B-3P	98.80	68.07	1204
HSA-MIR-513B-3P	98.76	68.12	1577
HSA-MIR-3908	98.75	67.31	1160
HSA-MIR-216B-3P	98.55	67.19	1223
HSA-MIR-1255B-2-3P	97.80	67.04	880

Literature-anchored findings (GeneRIF, showing 1)

• Data reveal a potential role of GASZ during embryonic germ cell development and provide a powerful in vitro system for dissecting the molecular pathways in early germ cell formation during embryogenesis. (PMID:23816659)

Cross-species orthologs

2 orthologs

Organism	Symbol	Gene ID
danio_rerio	asz1	ENSDARG00000004823
mus_musculus	Asz1	ENSMUSG00000010796

Paralogs (1): ANKRD34C (ENSG00000235711)

Protein

Protein identifiers

Ankyrin repeat, SAM and basic leucine zipper domain-containing protein 1 — Q8WWH4 (reviewed: Q8WWH4)

Alternative names: Ankyrin-like protein 1, Germ cell-specific ankyrin, SAM and basic leucine zipper domain-containing protein

All UniProt accessions (3): Q8WWH4, C9JP59, F8WDQ2

UniProt curated annotations — full annotation on UniProt →

Function. Plays a central role during spermatogenesis by repressing transposable elements and preventing their mobilization, which is essential for the germline integrity. Acts via the piRNA metabolic process, which mediates the repression of transposable elements during meiosis by forming complexes composed of piRNAs and Piwi proteins and governs the methylation and subsequent repression of transposons. Its association with pi-bodies suggests a participation in the primary piRNAs metabolic process. Required prior to the pachytene stage to facilitate the production of multiple types of piRNAs, including those associated with repeats involved in the regulation of retrotransposons. May act by mediating protein-protein interactions during germ cell maturation.

Subunit / interactions. Interacts with DDX4, PIWIL1, RANBP9 and TDRD1.

Subcellular location. Cytoplasm.

Tissue specificity. Expressed exclusively in the testis and ovary and at higher levels in the adult testis compared with the adult ovary.

Isoforms (2)

UniProt ID	Names	Canonical?
Q8WWH4-1	1	yes
Q8WWH4-2	2

RefSeq proteins (3): NP_001288750, NP_001288751, NP_570124* (*=MANE)

Domains & families (InterPro)

ID	Name	Type
IPR001660	SAM	Domain
IPR002110	Ankyrin_rpt	Repeat
IPR013761	SAM/pointed_sf	Homologous_superfamily
IPR036770	Ankyrin_rpt-contain_sf	Homologous_superfamily
IPR042650	Asz1_SAM	Domain

Pfam: PF00023, PF07647, PF12796

UniProt features (14 total): repeat 6, modified residue 3, chain 1, splice variant 1, sequence variant 1, domain 1, region of interest 1

Structure

Experimental structures (PDB)

0 structures.

Predicted structure (AlphaFold)

Model	pLDDT	Fraction very-high
AF-Q8WWH4-F1	66.83	0.32

Functional residue map

Curated UniProt residues grouped by drug-discovery relevance — catalytic, ligand-binding, modification, and mutation-validated positions. Source: UniProtKB sequence features.

Post-translational modifications (3): 18, 20, 17

Function

Pathways and Gene Ontology

Reactome pathways

3 pathways

ID	Pathway
R-HSA-5601884	PIWI-interacting RNA (piRNA) biogenesis
R-HSA-211000	Gene Silencing by RNA
R-HSA-74160	Gene expression (Transcription)

MSigDB gene sets: 58 (showing top): MIDORIKAWA_AMPLIFIED_IN_LIVER_CANCER, GRAESSMANN_APOPTOSIS_BY_SERUM_DEPRIVATION_UP, GOBP_MALE_GAMETE_GENERATION, GOBP_ORGANELLE_FISSION, GOBP_DEVELOPMENTAL_PROCESS_INVOLVED_IN_REPRODUCTION, GOBP_MEIOTIC_CELL_CYCLE_PROCESS, ACEVEDO_METHYLATED_IN_LIVER_CANCER_DN, GOBP_MEIOTIC_CELL_CYCLE, GOCC_RIBONUCLEOPROTEIN_GRANULE, GOCC_POLE_PLASM, GOBP_CELL_CYCLE_PROCESS, chr7q31, MIKKELSEN_IPS_HCP_WITH_H3_UNMETHYLATED, MIKKELSEN_ES_HCP_WITH_H3_UNMETHYLATED, MIKKELSEN_MEF_HCP_WITH_H3_UNMETHYLATED

GO Biological Process (6): male meiotic nuclear division (GO:0007140), spermatogenesis (GO:0007283), transposable element silencing (GO:0010526), cell differentiation (GO:0030154), regulatory ncRNA-mediated gene silencing (GO:0031047), meiotic cell cycle (GO:0051321)

GO Molecular Function (1): protein binding (GO:0005515)

GO Cellular Component (3): cytoplasm (GO:0005737), pi-body (GO:0071546), cytoplasmic ribonucleoprotein granule (GO:0036464)

Reactome top-level categories

Rollup of top-2 pathways:

Category	Pathways
Gene Silencing by RNA	1
Gene expression (Transcription)	1

GO top-level categories

Rollup of top GO terms by namespace:

Category	Terms
male gamete generation	2
meiotic nuclear division	2
negative regulation of gene expression	2
meiotic cell cycle	1
developmental process involved in reproduction	1
retrotransposition	1
cellular developmental process	1
cell cycle	1
sexual reproduction	1
reproductive process	1
binding	1
intracellular anatomical structure	1
cellular anatomical structure	1
P granule	1
cytoplasm	1
ribonucleoprotein granule	1

Protein interactions and networks

STRING

1764 interactions, top by confidence (×1000):

Protein A	Protein B	Partner UniProt	Score
ASZ1	CWC15	Q9P013	909
ASZ1	HPX	P02790	791
ASZ1	TSG101	Q99816	775
ASZ1	PLD6	Q8N2A8	732
ASZ1	PIWIL2	Q8TC59	717
ASZ1	SH2D3A	Q9BRG2	713
ASZ1	FAM3B	P58499	697
ASZ1	SH2D3C	Q8N5H7	697
ASZ1	FBL	P22087	680
ASZ1	AMBP	P00977	675
ASZ1	TDRD9	Q8NDG6	666
ASZ1	MOV10L1	Q9BXT6	656
ASZ1	TDRKH	Q9Y2W6	623
ASZ1	CTTNBP2	Q8WZ74	616
ASZ1	PPP1CA	P08129	603
ASZ1	MAEL	Q96JY0	603

IntAct

35 interactions, top by confidence:

A	B	Type	Score
rep	ASZ1	psi-mi:“MI:0915”(physical association)	0.600
NRAC	ASZ1	psi-mi:“MI:0915”(physical association)	0.560
TSPO2	ASZ1	psi-mi:“MI:0915”(physical association)	0.560
TMEM97	ASZ1	psi-mi:“MI:0915”(physical association)	0.560
SLC35A1	ASZ1	psi-mi:“MI:0915”(physical association)	0.560
BET1	ASZ1	psi-mi:“MI:0915”(physical association)	0.560
TMEM218	ASZ1	psi-mi:“MI:0915”(physical association)	0.560
ASZ1	LPAR3	psi-mi:“MI:0915”(physical association)	0.560
ASZ1	DEFB127	psi-mi:“MI:0915”(physical association)	0.560
TNFRSF10C	ASZ1	psi-mi:“MI:0915”(physical association)	0.560
SMIM1	ASZ1	psi-mi:“MI:0915”(physical association)	0.560
NRAC	ASZ1	psi-mi:“MI:0915”(physical association)	0.000
SMIM1	ASZ1	psi-mi:“MI:0915”(physical association)	0.000
DEFB127	ASZ1	psi-mi:“MI:0915”(physical association)	0.000
TSPO2	ASZ1	psi-mi:“MI:0915”(physical association)	0.000
TMEM97	ASZ1	psi-mi:“MI:0915”(physical association)	0.000
SLC35A1	ASZ1	psi-mi:“MI:0915”(physical association)	0.000
BET1	ASZ1	psi-mi:“MI:0915”(physical association)	0.000
TMEM218	ASZ1	psi-mi:“MI:0915”(physical association)	0.000
LPAR3	ASZ1	psi-mi:“MI:0915”(physical association)	0.000
TNFRSF10C	ASZ1	psi-mi:“MI:0915”(physical association)	0.000

BioGRID (14): ASZ1 (Two-hybrid), ASZ1 (Two-hybrid), ASZ1 (Two-hybrid), ASZ1 (Two-hybrid), ASZ1 (Two-hybrid), SLC35A1 (Two-hybrid), LPAR3 (Two-hybrid), TNFRSF10C (Two-hybrid), C14orf180 (Two-hybrid), SMIM1 (Two-hybrid), ASZ1 (Two-hybrid), MBLAC2 (Co-fractionation), ASZ1 (Affinity Capture-Western), TSG101 (Affinity Capture-Western)

ESM2 similar proteins: A0JM56, A0M8T3, A1X154, A4D7T3, A6NJI9, Q008S8, Q00PJ3, Q07DV3, Q07DX6, Q07DY6, Q07DZ7, Q07E17, Q07E30, Q07E43, Q09YH1, Q09YI3, Q09YJ5, Q09YK6, Q09YN0, Q0VD31, Q108U1, Q2IBB1, Q2IBB4, Q2IBE3, Q2IBF5, Q2IBG0, Q2QL84, Q2QLA4, Q2QLB5, Q2QLC6, Q2QLG0, Q2QLH1, Q32NR9, Q5PPX0, Q5ZLG9, Q6DCF6, Q6GQN5, Q6PJI9, Q6ZRR7, Q8BH70

Diamond homologs: A0M8T3, A1X154, A4D7T3, C9JTQ0, Q00PJ3, Q05823, Q05921, Q07DV3, Q07DX6, Q07DY6, Q07DZ7, Q07E17, Q07E30, Q07E43, Q09YH1, Q09YI3, Q09YJ5, Q09YK6, Q09YN0, Q108U1, Q2IBB1, Q2IBB4, Q2IBE3, Q2IBF5, Q2IBG0, Q2QL84, Q2QLA4, Q2QLB5, Q2QLC6, Q2QLG0, Q2QLH1, Q5E9N5, Q8IWZ3, Q8VD46, Q8WMX6, Q8WMX7, Q8WMX8, Q8WWH4, Q9FY48, Q9H078

SIGNOR signaling

0 interactions.

Disease & clinical

Clinical variants and AI predictions

ClinVar

67 variants total. Per-class counts are floors (≥ shown; pagination cap):

Classification	Count (floor)
Pathogenic	2
Likely pathogenic	0
Uncertain significance	58
Likely benign	5
Benign	1

Top pathogenic / likely-pathogenic (2)

Variant ID	HGVS	Classification
154392	GRCh38/hg38 7q31.1-31.31(chr7:114898882-117790123)x1	Pathogenic
3245669	NC_000007.13:g.(?116165117)(117307162_?)del	Pathogenic

SpliceAI

2032 predictions. Top by Δscore:

Variant	Effect	Δscore
7:117379932:TTTTA:T	donor_loss	1.0000
7:117379933:TTTAC:T	donor_loss	1.0000
7:117379934:TTAC:T	donor_loss	1.0000
7:117379935:TACC:T	donor_loss	1.0000
7:117379936:A:AC	donor_gain	1.0000
7:117379936:ACCT:A	donor_loss	1.0000
7:117379937:C:CC	donor_gain	1.0000
7:117380043:CCATT:C	acceptor_gain	1.0000
7:117380044:CATT:C	acceptor_gain	1.0000
7:117380044:CATTC:C	acceptor_gain	1.0000
7:117380045:ATT:A	acceptor_gain	1.0000
7:117380045:ATTC:A	acceptor_loss	1.0000
7:117380045:ATTCT:A	acceptor_gain	1.0000
7:117380046:TT:T	acceptor_gain	1.0000
7:117380048:C:CC	acceptor_gain	1.0000
7:117380048:CTAA:C	acceptor_loss	1.0000
7:117380053:C:CT	acceptor_gain	1.0000
7:117380054:A:T	acceptor_gain	1.0000
7:117380058:A:AC	acceptor_gain	1.0000
7:117380058:A:C	acceptor_gain	1.0000
7:117382141:TGAA:T	acceptor_gain	1.0000
7:117382145:C:CC	acceptor_gain	1.0000
7:117383075:CAA:C	acceptor_gain	1.0000
7:117383076:A:T	acceptor_gain	1.0000
7:117383083:T:C	acceptor_gain	1.0000
7:117383083:T:TC	acceptor_gain	1.0000
7:117384723:TACC:T	donor_loss	1.0000
7:117384727:T:TA	donor_gain	1.0000
7:117384858:AGCC:A	acceptor_loss	1.0000
7:117384859:GC:G	acceptor_gain	1.0000

AlphaMissense

3145 scored. Top likely-pathogenic:

Variant	Protein change	am_pathogenicity
7:117385780:G:T	A157D	0.976
7:117384811:A:G	L201S	0.975
7:117384859:G:T	A185D	0.974
7:117385783:G:T	A156D	0.972
7:117384848:C:G	A189P	0.971
7:117385781:C:G	A157P	0.970
7:117384847:G:T	A189E	0.965
7:117381042:A:G	L305P	0.964
7:117420246:A:C	C119W	0.963
7:117420248:A:G	C119R	0.961
7:117385715:C:G	D179H	0.956
7:117426847:A:G	L65P	0.956
7:117384845:C:G	A190P	0.955
7:117385784:C:G	A156P	0.955
7:117420196:A:G	L136P	0.955
7:117420199:A:G	L135P	0.950
7:117384748:G:T	A222E	0.949
7:117385747:A:T	L168H	0.949
7:117385777:C:G	R158P	0.946
7:117426884:C:G	A53P	0.944
7:117420199:A:C	L135R	0.943
7:117420199:A:T	L135Q	0.943
7:117384851:A:G	W188R	0.942
7:117384851:A:T	W188R	0.942
7:117384856:A:G	L186S	0.942
7:117385747:A:G	L168P	0.942
7:117420250:G:T	A118E	0.941
7:117385795:G:C	P152R	0.938
7:117384797:C:G	A206P	0.937
7:117385747:A:C	L168R	0.937

dbSNP variants (sampled 300 via entrez): RS1000024868 (7:117393524 A>AT), RS1000035626 (7:117417451 C>T), RS1000041895 (7:117393247 G>A,T), RS1000090450 (7:117385412 A>C), RS1000096403 (7:117391724 T>A), RS1000196073 (7:117375046 C>T), RS1000253003 (7:117378139 C>A,T), RS1000258323 (7:117386095 T>C), RS1000312470 (7:117378780 G>A), RS1000341997 (7:117427629 C>A,G,T), RS1000396830 (7:117370849 T>A), RS1000412438 (7:117378493 C>G,T), RS1000413087 (7:117421114 T>A), RS1000430522 (7:117370228 C>A,T), RS1000463953 (7:117427425 A>G)

Disease associations

OMIM: gene MIM:605797 | disease phenotypes: MIM:219700

GenCC curated gene-disease

Disease	Classification	Inheritance
male infertility with azoospermia or oligozoospermia due to single gene mutation	Moderate	Autosomal recessive

Mondo (2): cystic fibrosis (MONDO:0009061), (MONDO:0018393)

Orphanet (2): Male infertility with azoospermia or oligozoospermia due to single gene mutation (Orphanet:399805), Cystic fibrosis (Orphanet:586)

HPO phenotypes

0 total (0 of 0 shown, HPO-id order):

GWAS associations

9 associations (top):

Study	Trait	p-value
GCST002137_4	Waist circumference	4.000000e-06
GCST003739_1	Esophageal adenocarcinoma	6.000000e-09
GCST005196_149	Coronary artery disease	4.000000e-08
GCST005956_17	Waist-to-hip ratio adjusted for BMI	5.000000e-09
GCST005962_49	Waist-to-hip ratio adjusted for BMI x sex x age interaction (4df test)	3.000000e-07
GCST011461_13	Barrett’s esophagus or Esophageal adenocarcinoma	6.000000e-08
GCST011462_10	Barrett’s esophagus or esophageal adenocarcinoma x sex interaction (2df test)	4.000000e-08
GCST90020025_367	Waist-to-hip ratio adjusted for BMI	1.000000e-10
GCST90020027_1324	Waist-hip index	8.000000e-11

EFO canonical traits (3, from GWAS)

EFO ID	Trait name
EFO:0007788	BMI-adjusted waist-hip ratio
EFO:0008007	age at assessment
EFO:0008343	sex interaction measurement

MeSH disease descriptors (1)

Descriptor	Name	Tree numbers
D003550	Cystic Fibrosis	C06.689.202; C08.381.187; C16.320.190; C16.614.213

Drugs & pharmacology

Drug and pharmacology data

Is drug target: no

PharmGKB: 1 entry (VIP=true, CPIC=false)

CTD chemical–gene interactions

9 total (human), top 9 by PubMed support.

Chemical	Actions (top 5)	PubMed papers
pirinixic acid	affects binding, increases activity, increases expression	1
terbufos	increases methylation	1
perfluorooctanoic acid	increases expression	1
CGP 52608	increases reaction, affects binding	1
perfluorohexanesulfonic acid	increases expression	1
Benzo(a)pyrene	affects methylation	1
Fonofos	increases methylation	1
Parathion	increases methylation	1
Valproic Acid	increases methylation	1

Clinical trials (associated diseases)

300 trials via MONDO — disease-level, not drug-specific.

Trial	Phase	Status	Title
NCT00157690	PHASE4	COMPLETED	Study of Alendronate to Prevent and Treat Osteoporosis in Cystic Fibrosis Patients
NCT00208078	PHASE4	TERMINATED	Effect of Non-Invasive Ventilation in Cystic Fibrosis Patient With Chronic Respiratory Failure.
NCT00244270	PHASE4	COMPLETED	Cystic Fibrosis and Totally Implantable Vascular Access Devices
NCT00333385	PHASE4	TERMINATED	Continuous Versus Short Infusions of Ceftazidime in Cystic Fibrosis
NCT00411736	PHASE4	COMPLETED	Scandinavian Cystic Fibrosis Azithromycin Study
NCT00418470	PHASE4	TERMINATED	Prolonging the Duration of Peripheral Venous Catheters in Cystic Fibrosis People
NCT00431964	PHASE4	COMPLETED	Effect of Azithromycin on Lung Function in 6-18 Year-olds With Cystic Fibrosis (CF) Not Infected With P. Aeruginosa
NCT00434278	PHASE4	TERMINATED	A Trial of Pulmozyme Withdrawal on Exercise Tolerance in Cystic Fibrosis Subjects With Severe Lung Disease (TOPIC)
NCT00483769	PHASE4	COMPLETED	One Year Glargine Treatment in CFRD Children and Adolescents
NCT00528190	PHASE4	COMPLETED	Treatment of Aspergillus Fumigatus (a Fungal Infection) in Patients With Cystic Fibrosis
NCT00557089	PHASE4	COMPLETED	The Effect of rhDNase on Ventilation Inhomogeneity in Patients With Cystic Fibrosis
NCT00572975	PHASE4	COMPLETED	Malabsorption Blood Test:Toward a Novel Approach to Quantify Steatorrhea
NCT00680316	PHASE4	TERMINATED	A Study of Pulmozyme® (Dornase Alpha) in 3- to 5-Year-Old Patients With Cystic Fibrosis
NCT00685035	PHASE4	COMPLETED	Comparison of Airway Clearance Therapy in Cystic Fibrosis Using the Same VEST Therapy Device But With Different Settings
NCT00744250	PHASE4	TERMINATED	Intraduodenal Aspiration Study to Assess the Bioavailability of Oral Pancrecarb® Compared to Placebo Control
NCT00787917	PHASE4	TERMINATED	An Exploratory Study to Assess Multiple Doses of Omalizumab in Patients With Cystic Fibrosis Complicated by Acute Bronchopulmonary Aspergillosis (ABPA)
NCT00843817	PHASE4	COMPLETED	RhDNase and Biodistribution of PMN Serine Proteases in Cystic Fibrosis Sputum
NCT00890370	PHASE4	COMPLETED	Should Any One Airway Clearance Technique be Recommended for People With Cystic Fibrosis?
NCT00996424	PHASE4	TERMINATED	The Effect of Inhaled N-Acetylcysteine Compared to Normal Saline on Sputum Rheology and Lung Function
NCT01044719	PHASE4	UNKNOWN	Duration of Antibiotics in Infective Exacerbations of Cystic Fibrosis
NCT01100606	PHASE4	COMPLETED	A Study to Evaluate the Mode of Administration and Safety of EUR-1008 (APT-1008) in Infants 1 to 12 Months of Age
NCT01131507	PHASE4	COMPLETED	PR-018: An Open-Label, Safety Extension of Study PR-011
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NCT01323101	PHASE4	COMPLETED	Doxycycline Effects on Inflammation in Cystic Fibrosis
NCT01327703	PHASE4	COMPLETED	Control of Steatorrhea in Participants With Cystic Fibrosis and Exocrine Pancreatic Insufficiency
NCT01377792	PHASE4	COMPLETED	Study of Long-term Treatment With Hypertonic Saline in Patients With Cystic Fibrosis
NCT01400750	PHASE4	COMPLETED	Comparison of 2 Treatment Regimens for Eradication of P Aeruginosa Infection in Children With Cystic Fibrosis
NCT01429259	PHASE4	COMPLETED	Population Pharmacokinetics of Prolonged Infusion Meropenem in Cystic Fibrosis (CF) Children
NCT01608555	PHASE4	COMPLETED	Tobramycin 300 mg Once-a-day (o.d.) Aerosol in Adults With Cystic Fibrosis
NCT01667094	PHASE4	UNKNOWN	A Study Comparing Continuous Infusion Antibiotics to Standard Treatment for Lung Infections in Cystic Fibrosis
NCT01694069	PHASE4	TERMINATED	Continuous Infusion Piperacillin-tazobactam for the Treatment of Cystic Fibrosis
NCT01702415	PHASE4	WITHDRAWN	Zoledronic Acid in Cystic Fibrosis
NCT01712334	PHASE4	COMPLETED	A Study of the Comparable Efficacy and Safety of Pulmozyme (Dornase Alfa) Delivered by the eRapid Nebulizer System in Patients With Cystic Fibrosis
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NCT01880346	PHASE4	COMPLETED	Comparison of Absorption of Vitamin D in Cystic Fibrosis
NCT01882400	PHASE4	COMPLETED	Assessment of Response to Treatment of Osteoporosis With Oral Bisphosphonates in Patients With Muscular Dystrophy
NCT01937325	PHASE4	UNKNOWN	CPET in CF Patients With One G551D Mutation Taking VX770
NCT02015663	PHASE4	TERMINATED	Tobramycin Inhalation Powder (TIP) Administered Once Daily Continuously Versus TIP Administered BID in 28 Day on / 28 Day Off Cycles
NCT02048592	PHASE4	UNKNOWN	Impact of Immunonutrition on the Patients With Cystic Fibrosis