生物来源
rabbit
质量水平
偶联物
unconjugated
抗体形式
affinity isolated antibody
抗体产品类型
primary antibodies
克隆
polyclonal
产品线
Prestige Antibodies® Powered by Atlas Antibodies
形式
buffered aqueous glycerol solution
种属反应性
human
技术
immunoblotting: 0.04-0.4 μg/mL
immunohistochemistry: 1:500-1:1000
免疫原序列
GKSGTWWDEHLSEENVPFIKQLVSDEDKAQLASKLCPLKDEPWPIHPWEPGSFRVGLIALKLGMMPLWTKDGQKHVVTLLQVQDCHVLKYTSKENCNGK
UniProt登记号
运输
wet ice
储存温度
−20°C
靶向翻译后修饰
unmodified
基因信息
human ... MRPL3(11222)
一般描述
The gene MRPL3 (mitochondrial ribosomal protein L3) is mapped to human chromosome 3q22.1. It is nuclear encoded and is a large subunit protein of 39S subunit of 55S mitochondrial ribosome.
免疫原
mitochondrial ribosomal protein L3 recombinant protein epitope signature tag (PrEST)
应用
All Prestige Antibodies Powered by Atlas Antibodies are developed and validated by the Human Protein Atlas (HPA) project and as a result, are supported by the most extensive characterization in the industry.
The Human Protein Atlas project can be subdivided into three efforts: Human Tissue Atlas, Cancer Atlas, and Human Cell Atlas. The antibodies that have been generated in support of the Tissue and Cancer Atlas projects have been tested by immunohistochemistry against hundreds of normal and disease tissues and through the recent efforts of the Human Cell Atlas project, many have been characterized by immunofluorescence to map the human proteome not only at the tissue level but now at the subcellular level. These images and the collection of this vast data set can be viewed on the Human Protein Atlas (HPA) site by clicking on the Image Gallery link. We also provide Prestige Antibodies® protocols and other useful information.
The Human Protein Atlas project can be subdivided into three efforts: Human Tissue Atlas, Cancer Atlas, and Human Cell Atlas. The antibodies that have been generated in support of the Tissue and Cancer Atlas projects have been tested by immunohistochemistry against hundreds of normal and disease tissues and through the recent efforts of the Human Cell Atlas project, many have been characterized by immunofluorescence to map the human proteome not only at the tissue level but now at the subcellular level. These images and the collection of this vast data set can be viewed on the Human Protein Atlas (HPA) site by clicking on the Image Gallery link. We also provide Prestige Antibodies® protocols and other useful information.
生化/生理作用
Mutation in the MRPL3 (mitochondrial ribosomal protein L3) gene causes hypertrophic cardiomyopathy. It also results in psychomotor retardation, particularly due to defects in mitochondrial translation. In addition, mutation in MRPL3 might also be associated with autosomal dominant non-syndromic hearing loss (DFNA18).
特点和优势
Prestige Antibodies® are highly characterized and extensively validated antibodies with the added benefit of all available characterization data for each target being accessible via the Human Protein Atlas portal linked just below the product name at the top of this page. The uniqueness and low cross-reactivity of the Prestige Antibodies® to other proteins are due to a thorough selection of antigen regions, affinity purification, and stringent selection. Prestige antigen controls are available for every corresponding Prestige Antibody and can be found in the linkage section.
Every Prestige Antibody is tested in the following ways:
Every Prestige Antibody is tested in the following ways:
- IHC tissue array of 44 normal human tissues and 20 of the most common cancer type tissues.
- Protein array of 364 human recombinant protein fragments.
联系
Corresponding Antigen APREST84658
外形
Solution in phosphate buffered saline, pH 7.2, containing 40% glycerol and 0.02% sodium azide.
法律信息
Prestige Antibodies is a registered trademark of Merck KGaA, Darmstadt, Germany
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WGK
WGK 1
闪点(°F)
Not applicable
闪点(°C)
Not applicable
法规信息
常规特殊物品
Exome sequencing identifies MRPL3 mutation in mitochondrial cardiomyopathy.
Galmiche L, et al.
Human Mutation, 32, 1225-1231 (2011)
Thomas W O'Brien et al.
Gene, 354, 147-151 (2005-05-24)
The ancestral mitochondrial ribosome (70S) underwent major structural remodeling during the evolution of mammalian mitochondrial ribosomes (55S). Despite the loss of nearly half their RNA, 55S ribosomes are actually larger than bacterial ribosomes because of all the extra proteins they
Lindsey Van Haute et al.
Nature communications, 14(1), 1009-1009 (2023-02-24)
Mutations in the mitochondrial or nuclear genomes are associated with a diverse group of human disorders characterized by impaired mitochondrial respiration. Within this group, an increasing number of mutations have been identified in nuclear genes involved in mitochondrial RNA biology.
Aurelio Reyes et al.
PLoS genetics, 16(7), e1008923-e1008923 (2020-08-01)
Mitochondrial translation defects can be due to mutations affecting mitochondrial- or nuclear-encoded components. The number of known nuclear genes involved in mitochondrial translation has significantly increased in the past years. RCC1L (WBSCR16), a putative GDP/GTP exchange factor, has recently been
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