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The molecular defect in a protein (CRA) found in gamma-1 heavy chain disease, and its genetic implications.在γ-1重链病中发现的一种蛋白质(CRA)的分子缺陷及其遗传学意义。
Proc Natl Acad Sci U S A. 1971 Jan;68(1):187-91. doi: 10.1073/pnas.68.1.187.
2
Molecular defect in a gamma-2 heavy chain.γ-2重链中的分子缺陷
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"Alpha chain disease" protein def: internal deletion of a human immunoglobulin A1 heavy chain.“α链病”蛋白定义:人免疫球蛋白A1重链的内部缺失。
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4
Partial deletion in the heavy chain disease protein ZUC.重链病蛋白ZUC中的部分缺失。
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Protein Hal: partial deletion of a " " immunoglobulin gene(s) and apparent reinitiation at an internal AUG codon.蛋白质Hal:一个“ ”免疫球蛋白基因的部分缺失以及在内源AUG密码子处明显的重新起始。
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Human immunoglobulin subclasses. Partial amino acid sequence of the constant region of a gamma 4 chain.人免疫球蛋白亚类。γ4链恒定区的部分氨基酸序列。
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引用本文的文献

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The gamma 1 heavy-chain disease FOR protein is present in two molecular forms.γ1重链病的FOR蛋白以两种分子形式存在。
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Immunology. 1984 Jan;51(1):17-25.
3
N-terminal amino-acid sequence of African lungfish immunoglobulin light chains.非洲肺鱼免疫球蛋白轻链的N端氨基酸序列。
Proc Natl Acad Sci U S A. 1971 Oct;68(10):2321-4. doi: 10.1073/pnas.68.10.2321.
4
"Alpha chain disease" protein def: internal deletion of a human immunoglobulin A1 heavy chain.“α链病”蛋白定义:人免疫球蛋白A1重链的内部缺失。
Proc Natl Acad Sci U S A. 1974 Mar;71(3):974-8. doi: 10.1073/pnas.71.3.974.
5
Protein Hal: partial deletion of a " " immunoglobulin gene(s) and apparent reinitiation at an internal AUG codon.蛋白质Hal:一个“ ”免疫球蛋白基因的部分缺失以及在内源AUG密码子处明显的重新起始。
Proc Natl Acad Sci U S A. 1973 Apr;70(4):1073-7. doi: 10.1073/pnas.70.4.1073.
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7
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8
Production of monoclonal antibodies to lactate dehydrogenase (LDH) isoenzymes for immunohistochemical study on fixed tissue section.制备用于固定组织切片免疫组织化学研究的乳酸脱氢酶(LDH)同工酶单克隆抗体。
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gamma- and mu-Heavy chain diseases and related disorders.γ和μ重链病及相关疾病
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10
A new immunoglobulin variant: gamma3 heavy chain disease protein CHI.一种新的免疫球蛋白变体:γ3重链病蛋白CHI
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STRUCTURAL STUDIES OF HUMAN 7S GAMMA-GLOBULIN (G IMMUNOGLOBULIN). FURTHER OBSERVATIONS OF A NATURALLY OCCURRING PROTEIN RELATED TO THE CRYSTALLIZABLE (FAST) FRAGMENT.人7Sγ球蛋白(G免疫球蛋白)的结构研究。与可结晶(快速)片段相关的一种天然存在蛋白质的进一步观察。
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Separation of dansyl-amino acids by polyamide layer chromatography.用聚酰胺薄层层析法分离丹磺酰氨基酸。
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Characterization of a human macroglobulin. II. Distribution of the disulfide bonds.一种人巨球蛋白的特性。II. 二硫键的分布
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Structural studies of immunoglobulin G.免疫球蛋白G的结构研究。
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Heavy-chain diseases.重链病
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The covalent structure of an entire gammaG immunoglobulin molecule.完整的γG免疫球蛋白分子的共价结构。
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Intrachain disulphide bridges in immunoglobulin G heavy chains. The Fc fragment.免疫球蛋白G重链中的链内二硫键。Fc片段。
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10
Sense and nonsense in the genetic code. Three exceptional triplets can serve as both chain-terminating signals and amino acid codons.遗传密码中的意义与无意义。三个特殊的三联体既可以作为链终止信号,也可以作为氨基酸密码子。
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在γ-1重链病中发现的一种蛋白质(CRA)的分子缺陷及其遗传学意义。

The molecular defect in a protein (CRA) found in gamma-1 heavy chain disease, and its genetic implications.

作者信息

Franklin E C, Frangione B

出版信息

Proc Natl Acad Sci U S A. 1971 Jan;68(1):187-91. doi: 10.1073/pnas.68.1.187.

DOI:10.1073/pnas.68.1.187
PMID:4100047
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC391192/
Abstract

A gamma1 protein, designated CRA, found in heavy chain disease contains three inter-heavy disulfide bridges instead of the two normally found in gamma1 immunoglobulin heavy chains. Almost the entire Fd fragment is missing. The NH(2) terminal region is heterogeneous and contains carbohydrate; after 11 residues that do not resemble any of the known heavy-chain variable-region subclasses, normal synthesis seems to be resumed at the same amino acid residue as in another heavy-chain disease protein (ZUC). This finding raises the possibility that glutamic acid at position 216 represents the beginning of the Fe region, synthesized under the direction of another gene.

摘要

在重链病中发现的一种γ1蛋白,命名为CRA,含有三个重链间二硫键,而不是γ1免疫球蛋白重链中通常存在的两个。几乎整个Fd片段缺失。NH(2)末端区域具有异质性且含有碳水化合物;在11个与任何已知重链可变区亚类都不相似的残基之后,正常合成似乎在与另一种重链病蛋白(ZUC)相同的氨基酸残基处恢复。这一发现增加了216位谷氨酸代表在另一个基因指导下合成的Fe区域起始点的可能性。