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AB162126

重组人 MMADHC 蛋白 (GST tag N-Terminus)

Recombinant Human MMADHC protein (GST tag N-Terminus)

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Recombinant Human MMADHC protein (GST tag N-Terminus) is a Human Full Length protein, in the 1 to 296 aa range, expressed in Wheat germ, suitable for ELISA, WB.

查看别名

C2orf25, CL25022, HSPC161, My011, MMADHC, Cobalamin trafficking protein CblD, CblD

1 Images
SDS-PAGE - Recombinant Human MMADHC protein (GST tag N-Terminus) (AB162126)
  • SDS-PAGE

Unknown

SDS-PAGE - Recombinant Human MMADHC protein (GST tag N-Terminus) (AB162126)

ab162126 on a 12.5% SDS-PAGE stained with Coomassie Blue.

关键信息

表达系统

Wheat germ

标签

GST tag N-Terminus

应用

WB, ELISA

applications

生物活性

No

访问

Q9H3L0

不含动物源

No

不含载体蛋白

No

种属

Human

存储溶液

pH: 8 Constituents: 0.79% Tris HCl, 0.31% Glutathione

storage-buffer

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搭配使用产品

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SDS-PAGE - Recombinant Mouse OSTM1 protein (His tag) (AB277009)

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Proteins & Peptides

AB162220

Recombinant Human MCJ protein (GST tag N-Terminus)

SDS-PAGE - Recombinant Human MCJ protein (GST tag N-Terminus) (AB162220)

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Proteins & Peptides

AB162150

Recombinant Human TMEM97 protein (GST tag N-Terminus)

SDS-PAGE - Recombinant Human TMEM97 protein (GST tag N-Terminus) (AB162150)

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Proteins & Peptides

AB161869

Recombinant Human SPS2L/SPATS2L protein (GST tag N-Terminus)

SDS-PAGE - Recombinant Human SPS2L/SPATS2L protein (GST tag N-Terminus) (AB161869)

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  • 0
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反应性数据

{ "title": "Reactivity Data", "filters": { "stats": ["", "Reactivity", "Dilution Info", "Notes"] }, "values": { "ELISA": { "reactivity":"TESTED_AND_REACTS", "dilution-info":"", "notes":"<p></p>" }, "WB": { "reactivity":"TESTED_AND_REACTS", "dilution-info":"", "notes":"<p></p>" } } }
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产品详情

This product was previously labelled as C2ORF25.
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序列信息

[{"linker":null,"sequence":"MANVLCNRARLVSYLPGFCSLVKRVVNPKAFSTAGSSGSDESHVAAAPPDICSRTVWPDETMGPFGPQDQRFQLPGNIGFDCHLNGTASQKKSLVHKTLPDVLAEPLSSERHEFVMAQYVNEFQGNDAPVEQEINSAETYFESARVECAIQTCPELLRKDFESLFPEVANGKLMILTVTQKTKNDMTVWSEEVEIEREVLLEKFINGAKEICYALRAEGYWADFIDPSSGLAFFGPYTNNTLFETDERYRHLGFSVDDLGCCKVIRHSLWGTHVVVGSIFTNATPDSHIMKKLSGN","proteinLength":"Full Length","predictedMolecularWeight":null,"actualMolecularWeight":null,"aminoAcidEnd":296,"aminoAcidStart":1,"nature":"Recombinant","expressionSystem":"Wheat germ","accessionNumber":"Q9H3L0","tags":[{"tag":"GST","terminus":"N-Terminus"}]}]
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性能和储存信息

形式
Liquid
纯化工艺
Proprietary technique
运输条件
Dry Ice
推荐的短期储存条件
-80°C
推荐的长期储存条件
-80°C
分装信息
Upon delivery aliquot
储存信息
Avoid freeze / thaw cycle
False
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补充信息

This supplementary information is collated from multiple sources and compiled automatically.

MMADHC also known as methylmalonic aciduria and homocystinuria cblD type is a protein involved in the metabolism of vitamin B12. It has a molecular weight of approximately 32 kDa. MMADHC is expressed in various tissues including liver kidney and brain. It acts as a chaperone-like factor that helps direct cobalamin into the cytosol and mitochondrian important for vitamin B12-associated reactions.
Biological function summary

MMADHC plays a role in the intracellular processing of vitamin B12. It is not part of a complex but interacts closely with the mitochondrial methylmalonyl-CoA mutase and cytosolic methionine synthase pathways. By assisting in cobalamin transport it enables the conversion of methylmalonic acid to succinyl-CoA and homocysteine to methionine therefore supporting essential cellular reactions.

Pathways

MMADHC contributes to the cobalamin metabolic pathway and the methionine metabolism pathway. In the cobalamin pathway it works alongside proteins like MMACHC and MCM to facilitate vitamin B12 metabolism. It ensures that vitamin B12 reaches its target enzymes efficiently which is vital for methylation processes and energy production pathways in the mitochondria.

Mutations in MMADHC are linked to methylmalonic aciduria with homocystinuria cblD type. This disorder results from improper processing of vitamin B12 and often leads to neurological and metabolic complications. The protein is associated with disorders involving defective cobalamin metabolism alongside MMACHC highlighting its key role in maintaining normal vitamin B12-dependent enzyme function.
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常规信息

功能

Involved in cobalamin metabolism and trafficking (PubMed : 18385497, PubMed : 23415655, PubMed : 24722857, PubMed : 26364851). Plays a role in regulating the biosynthesis and the proportion of two coenzymes, methylcob(III)alamin (MeCbl) and 5'-deoxyadenosylcobalamin (AdoCbl) (PubMed : 18385497, PubMed : 23415655, PubMed : 24722857). Promotes oxidation of cob(II)alamin bound to MMACHC (PubMed : 26364851). The processing of cobalamin in the cytosol occurs in a multiprotein complex composed of at least MMACHC, MMADHC, MTRR (methionine synthase reductase) and MTR (methionine synthase) which may contribute to shuttle safely and efficiently cobalamin towards MTR in order to produce methionine (PubMed : 27771510).

亚细胞定位

Mitochondrion

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产品实验方案

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靶点信息

Involved in cobalamin metabolism and trafficking (PubMed : 18385497, PubMed : 23415655, PubMed : 24722857, PubMed : 26364851). Plays a role in regulating the biosynthesis and the proportion of two coenzymes, methylcob(III)alamin (MeCbl) and 5'-deoxyadenosylcobalamin (AdoCbl) (PubMed : 18385497, PubMed : 23415655, PubMed : 24722857). Promotes oxidation of cob(II)alamin bound to MMACHC (PubMed : 26364851). The processing of cobalamin in the cytosol occurs in a multiprotein complex composed of at least MMACHC, MMADHC, MTRR (methionine synthase reductase) and MTR (methionine synthase) which may contribute to shuttle safely and efficiently cobalamin towards MTR in order to produce methionine (PubMed : 27771510).
See full target information MMADHC
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