LYRM4 Antibody, FITC conjugated

Code CSB-PA875729LC01HU
Size US$166
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Product Details

Full Product Name
Rabbit anti-Homo sapiens (Human) LYRM4 Polyclonal antibody
Uniprot No.
Target Names
LYRM4
Alternative Names
BC034664 antibody; C6orf149 antibody; CGI 203 antibody; Chromosome 6 open reading frame 149 antibody; Gm903 antibody; Homolog of yeast Isd11 antibody; ISD11 antibody; LYR motif containing 4 antibody; LYR motif-containing protein 4 antibody; LYRM4 antibody; LYRM4_HUMAN antibody; MGC38622 antibody; Mitochondrial matrix Nfs1 interacting protein antibody
Raised in
Rabbit
Species Reactivity
Human
Immunogen
Recombinant Human LYR motif-containing protein 4 protein (1-91AA)
Immunogen Species
Homo sapiens (Human)
Conjugate
FITC
Clonality
Polyclonal
Isotype
IgG
Purification Method
>95%, Protein G purified
Concentration
It differs from different batches. Please contact us to confirm it.
Buffer
Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
Form
Liquid
Troubleshooting and FAQs
Storage
Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
Lead Time
Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

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Target Background

Function
Required for nuclear and mitochondrial iron-sulfur protein biosynthesis.
Gene References into Functions
  1. analysis of the NFS1-ISD11-ACP (SDA) complex forms the core of the iron-sulfur (Fe-S) assembly complex and associates with assembly proteins ISCU2, frataxin (FXN), and ferredoxin to synthesize Fe-S clusters PMID: 28634302
  2. Human Isd11 (ISD11) is a helical protein which exists in solution as an equilibrium between monomer, dimeric and tetrameric species when in the absence of human Nfs1 (NFS1). Recombinant ISD11 expressed in E. coli co-purifies with the bacterial orthologue of NFS1, IscS. PMID: 27427956
  3. The NFS1/ISD11 complex further interacts with scaffold protein ISCU and regulator protein frataxin, thereby forming a quaternary complex for Fe-S cluster formation. PMID: 28271877
  4. Molecular dynamics flexible fitting of protein structures docked into the EM map of the model revealed a [FXN(42-210)]24.[NFS1]24.[ISD11]24.[ISCU]24 complex, consistent with the measured 1:1:1:1 stoichiometry of its four components. PMID: 27519411
  5. Our findings highlight that the ISD11 R68A/R68L mutation display reduced affinity to form a stable subcomplex with NFS1, and thereby fails to prevent NFS1 aggregation resulting in impairment of the Fe-S cluster biogenesis PMID: 26342079
  6. Homozygous mutation in LYRM4 was reported in two patients with combined oxidative phosphorylation deficiency. PMID: 23814038
  7. LYRM4 downregulation may be one of the mechanisms involved in inefficient oxidative phosphorylation and oxidative stress, increasingly recognised as contributors to schizophrenia pathogenesis. PMID: 21968932
  8. Strong candidate gene for mitochondrial disease, based on recessive mutations detected in infantile patients PMID: 22277967
  9. Frataxin interacts with ISD11 and multiple mitochondrial chaperones. PMID: 17331979
  10. ISD11 is important in the biogenesis of Fe-S clusters in mammalian cells. PMID: 19454487

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Involvement in disease
Combined oxidative phosphorylation deficiency 19 (COXPD19)
Subcellular Location
Mitochondrion. Nucleus.
Protein Families
Complex I LYR family
Tissue Specificity
Reduced mRNA levels in Friedreich ataxia patients.
Database Links

HGNC: 21365

OMIM: 613311

KEGG: hsa:57128

STRING: 9606.ENSP00000443900

UniGene: Hs.387755

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