Tag Content
SG ID
SG00000107 
UniProt Accession
Theoretical PI
5.64  
Molecular Weight
80886 Da  
Genbank Nucleotide ID
Genbank Protein ID
 
Gene Name
mlh1 
Gene Synonyms/Alias
 
Protein Name
 
Protein Synonyms/Alias
SubName: Uncharacterized protein 
Organism
Danio rerio (Zebrafish) (Brachydanio rerio) 
NCBI Taxonomy ID
7955 
Chromosome Location
chr:13;43255387-43270620;1
View in Ensembl genome browser  
Function in Stage
Function in Cell Type
Description
Temporarily unavailable 
The information of related literatures
1. M. C. Leal, H. Feitsma, E. Cuppen, L. R. Franca and R. W. Schulz (2008) Completion of meiosis in male zebrafish (Danio rerio) despite lack of DNA mismatch repair gene mlh1. Cell Tissue Res 332(1): 133-9. 

Abstract
Mlh1 is a member of DNA mismatch repair (MMR) machinery and is also essential for the stabilization of crossovers during the first meiotic division. Recently, we have shown that zebrafish mlh1 mutant males are completely infertile because of a block in metaphase I, whereas females are fertile but have aneuploid progeny. When studying fertility in males in a two-fold more inbred background, we have however observed low numbers of fertilized eggs (approximately 0.4%). Histological examination of the testis has revealed that all spermatogenic stages prior to spermatids (spermatogonia, primary spermatocytes, and secondary spermatocytes) are significantly increased in the mutant, whereas the total weight of spermatids and spermatozoa is highly decreased (1.8 mg in wild-type vs. 0.1 mg in mutants), a result clearly different from our previous study in which outbred males lack secondary spermatocytes or postmeiotic cells. Thus, a delay of both meiotic divisions occurs rather than complete arrest during meiosis I in these males. Eggs fertilized with mutant sperm develop as malformed embryos and are aneuploid making this male phenotype much more similar to that previously described in the mutant females. Therefore, crossovers are still essential for proper meiosis, but meiotic cell divisions can progress without it, suggesting that this mutant is a suitable model for studying the cellular mechanisms of completing meiosis without crossover stabilization. PMID: [18247060] 

2. H. Feitsma, M. C. Leal, P. B. Moens, E. Cuppen and R. W. Schulz (2007) Mlh1 deficiency in zebrafish results in male sterility and aneuploid as well as triploid progeny in females. Genetics 175(4): 1561-9. 

Abstract
In most eukaryotes, recombination of homologous chromosomes during meiosis is necessary for proper chromosome pairing and subsequent segregation. The molecular mechanisms of meiosis are still relatively unknown, but numerous genes are known to be involved, among which are many mismatch repair genes. One of them, mlh1, colocalizes with presumptive sites of crossing over, but its exact action remains unclear. We studied meiotic processes in a knockout line for mlh1 in zebrafish. Male mlh1 mutants are sterile and display an arrest in spermatogenesis at metaphase I, resulting in increased testis weight due to accumulation of prophase I spermatocytes. In contrast, females are fully fertile, but their progeny shows high rates of dysmorphology and mortality within the first days of development. SNP-based chromosome analysis shows that this is caused by aneuploidy, resulting from meiosis I chromosomal missegregation. Surprisingly, the small percentage of progeny that develops normally has a complete triploid genome, consisting of both sets of maternal and one set of paternal chromosomes. As adults, these triploid fish are infertile males with wild-type appearance. The frequency of triploid progeny of mlh1 mutant females is much higher than could be expected for random chromosome segregation. Together, these results show that multiple solutions exist for meiotic crossover/segregation problems. PMID: [17237513] 

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Figures for illustrating the function of this protein/gene
Ref: H. Feitsma, M. C. Leal, P. B. Moens, E. Cuppen and R. W. Schulz (2007) Mlh1 deficiency in zebrafish results in male sterility and aneuploid as well as triploid progeny in females. Genetics 175(4): 1561-9. PMID: [17237513]
Ref: H. Feitsma, M. C. Leal, P. B. Moens, E. Cuppen and R. W. Schulz (2007) Mlh1 deficiency in zebrafish results in male sterility and aneuploid as well as triploid progeny in females. Genetics 175(4): 1561-9. PMID: [17237513]
Ref: H. Feitsma, M. C. Leal, P. B. Moens, E. Cuppen and R. W. Schulz (2007) Mlh1 deficiency in zebrafish results in male sterility and aneuploid as well as triploid progeny in females. Genetics 175(4): 1561-9. PMID: [17237513]
Ref: H. Feitsma, M. C. Leal, P. B. Moens, E. Cuppen and R. W. Schulz (2007) Mlh1 deficiency in zebrafish results in male sterility and aneuploid as well as triploid progeny in females. Genetics 175(4): 1561-9. PMID: [17237513]
Function
 
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Subcellular Location
 
Tissue Specificity
 
Gene Ontology
GO IDGO termEvidence
GO:0005634 C:nucleus IEA:InterPro.
GO:0005524 F:ATP binding IEA:InterPro.
GO:0030983 F:mismatched DNA binding IEA:InterPro.
GO:0006298 P:mismatch repair IEA:InterPro.
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Interpro
IPR003594;    ATPase-like_ATP-bd.
IPR002099;    DNA_mismatch_repair.
IPR013507;    DNA_mismatch_repair_C.
IPR014762;    DNA_mismatch_repair_CS.
IPR011186;    DNA_mismatch_repair_MLH1.
IPR014763;    DNA_mismatch_repair_N.
IPR020568;    Ribosomal_S5_D2-typ_fold.
IPR014721;    Ribosomal_S5_D2-typ_fold_subgr.
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Pfam
PF01119;    DNA_mis_repair;    1.
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SMART
SM00387;    HATPase_c;    1.
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PROSITE
PS00058;    DNA_MISMATCH_REPAIR_1;    1.
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PRINTS
Created Date
18-Oct-2012 
Record Type
Experiment identified 
Protein sequence Annotation
Nucleotide Sequence
Length: 158912 bp   Go to nucleotide: FASTA
Protein Sequence
Length: 724 bp   Go to amino acid: FASTA
The verified Protein-Protein interaction information
UniProt
Gene Symbol Ref Databases
Other Protein-Protein interaction resources
String database  
View Microarray data
Temporarily unavailable 
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