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AB204313

Anti-MMADHC抗体

Anti-MMADHC antibody

4

(2 Reviews)

|

(1 Publication)

Rabbit Polyclonal MMADHC antibody. Suitable for IHC-P, WB and reacts with Human samples. Cited in 1 publication. Immunogen corresponding to Recombinant Fragment Protein within Human MMADHC aa 50-200.

查看别名

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

5 Images
Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-MMADHC antibody (AB204313)
  • IHC-P

Supplier Data

Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-MMADHC antibody (AB204313)

Immunohistochemical analysis of human liver tissue labeling MMADHC with ab204313 at 1/200 dilution.

Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-MMADHC antibody (AB204313)
  • IHC-P

Supplier Data

Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-MMADHC antibody (AB204313)

Immunohistochemical analysis of human testis tissue labeling MMADHC with ab204313 at 1/200 dilution.

Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-MMADHC antibody (AB204313)
  • IHC-P

Supplier Data

Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-MMADHC antibody (AB204313)

Immunohistochemical analysis of human prostate tissue labeling MMADHC with ab204313 at 1/200 dilution.

Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-MMADHC antibody (AB204313)
  • IHC-P

Supplier Data

Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-MMADHC antibody (AB204313)

Immunohistochemical analysis of human placenta tissue labeling MMADHC with ab204313 at 1/200 dilution.

Western blot - Anti-MMADHC antibody (AB204313)
  • WB

Supplier Data

Western blot - Anti-MMADHC antibody (AB204313)

All lanes:

Western blot - Anti-MMADHC antibody (ab204313) at 0.4 µg/mL

All lanes:

K562 cells

Predicted band size: 33 kDa

false

关键信息

宿主种属

Rabbit

克隆

Polyclonal

亚型

IgG

不含载体蛋白

No

反应种属

Human

应用

WB, IHC-P

applications

免疫原

Recombinant Fragment Protein within Human MMADHC aa 50-200. The exact immunogen used to generate this antibody is proprietary information.

Q9H3L0

反应性数据

{ "title": "Reactivity Data", "filters": { "stats": ["", "Species", "Dilution Info", "Notes"], "tabs": { "all-applications": {"fullname" : "All Applications", "shortname": "All Applications"}, "IHCP" : {"fullname" : "Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections)", "shortname":"IHC-P"}, "WB" : {"fullname" : "Western blot", "shortname":"WB"} }, "product-promise": { "all": "all", "testedAndGuaranteed": "tested", "guaranteed": "expected", "predicted": "predicted", "notRecommended": "not-recommended" } }, "values": { "Human": { "IHCP-species-checked": "testedAndGuaranteed", "IHCP-species-dilution-info": "1/200 - 1/500", "IHCP-species-notes": "<p></p> Perform heat-mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.", "WB-species-checked": "testedAndGuaranteed", "WB-species-dilution-info": "0.04-0.4 µg/mL", "WB-species-notes": "<p></p>" }, "Mouse": { "IHCP-species-checked": "predicted", "IHCP-species-dilution-info": "", "IHCP-species-notes": "", "WB-species-checked": "predicted", "WB-species-dilution-info": "", "WB-species-notes": "" }, "Rat": { "IHCP-species-checked": "predicted", "IHCP-species-dilution-info": "", "IHCP-species-notes": "", "WB-species-checked": "predicted", "WB-species-dilution-info": "", "WB-species-notes": "" } } }

性能和储存信息

形式
Liquid
纯化工艺
Affinity purification Immunogen
存储溶液
pH: 7.2 Preservative: 0.02% Sodium azide Constituents: PBS, 40% Glycerol (glycerin, glycerine)
运输条件
Blue Ice
推荐的短期储存时间
1-2 weeks
推荐的短期储存条件
+4°C
推荐的长期储存条件
-20°C
分装信息
Upon delivery aliquot
储存信息
Avoid freeze / thaw cycle

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.

产品实验方案

For this product, it's our understanding that no specific protocols are required. You can visit:

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

文献 (1)

Recent publications for all applications. Explore the full list and refine your search

Genetics in medicine open 2:100841 PubMed39669623

2023

Clinical study of ferredoxin-reductase-related mitochondriopathy: Genotype-phenotype correlation and proposal of ancestry-based carrier screening in the Mexican population.

Applications

Unspecified application

Species

Unspecified reactive species

Teresa Campbell,Jesse Slone,Hallie Metzger,Wensheng Liu,Stephanie Sacharow,Amy Yang,Mariya Moosajee,Chiara La Morgia,Valerio Carelli,Flavia Palombo,Matthew A Lines,A Micheil Innes,Rebecca J Levy,Derek Neilson,Nicola Longo,Taosheng Huang
View all publications

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