MMADHC Antibody

Datasheet

Code	CSB-PA875665LA01HU
Size	US$166
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Image	IHC image of CSB-PA875665LA01HU diluted at 1:300 and staining in paraffin-embedded human glioma performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system. IHC image of CSB-PA875665LA01HU diluted at 1:300 and staining in paraffin-embedded human prostate cancer performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system. Immunofluorescence staining of HepG2 cells with CSB-PA875665LA01HU at 1:100, counter-stained with DAPI. The cells were fixed in 4% formaldehyde, permeabilized using 0.2% Triton X-100 and blocked in 10% normal Goat Serum. The cells were then incubated with the antibody overnight at 4°C. The secondary antibody was Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L).
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Target Background

Product Details

Full Product Name

Rabbit anti-Homo sapiens (Human) MMADHC Polyclonal antibody

Uniprot No.

Q9H3L0

Target Names

MMADHC

Alternative Names

C2orf25 antibody; cblD antibody; Chromosome 2 open reading frame 25 antibody; CL25022 antibody; Methylmalonic aciduria (cobalamin deficiency) cblD type; with homocystinuria antibody; Methylmalonic aciduria and homocystinuria type D protein antibody; methylmalonic aciduria and homocystinuria type D protein; mitochondrial antibody; mitochondrial antibody; MMAD_HUMAN antibody; Mmadhc antibody; Protein C2orf25; mitochondrial antibody

Raised in

Rabbit

Species Reactivity

Human

Immunogen

Recombinant Human Methylmalonic aciduria and homocystinuria type D protein, mitochondrial protein (26-142AA)

Immunogen Species

Homo sapiens (Human)

Conjugate

Non-conjugated

The MMADHC Antibody (Product code: CSB-PA875665LA01HU) is Non-conjugated. For MMADHC Antibody with conjugates, please check the following table.

Conjugate	Product Code	Product Name	Application
HRP	CSB-PA875665LB01HU	MMADHC Antibody, HRP conjugated	ELISA
FITC	CSB-PA875665LC01HU	MMADHC Antibody, FITC conjugated
Biotin	CSB-PA875665LD01HU	MMADHC Antibody, Biotin conjugated	ELISA

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

Tested Applications

ELISA, IHC, IF

Recommended Dilution

Application	Recommended Dilution
IHC	1:200-1:500
IF	1:50-1:200

Protocols

ELISA Protocol
Immunohistochemistry (IHC) Protocol
Immunofluorescence (IF) Protocol

Troubleshooting and FAQs

Antibody 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.

Customer Reviews and Q&A

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Target Background

Function

Involved in cobalamin metabolism and trafficking. Plays a role in regulating the biosynthesis and the proportion of two coenzymes, methylcob(III)alamin (MeCbl) and 5'-deoxyadenosylcobalamin (AdoCbl). Promotes oxidation of cob(II)alamin bound to MMACHC. 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.

Gene References into Functions

analysis of crystal structure of the globular C-terminal domain of human CblD, which is sufficient for its interaction with MMADHC or CblC, and for supporting the cytoplasmic cobalamin trafficking pathway PMID: 26364851
specific regions of MMADHC are involved in differential regulation of adenosylcobalamin and methylcobalamin synthesis PMID: 24722857
MMADHC mutations are associated with methylmalonic aciduria and homocystinuria. PMID: 22156578
mutations in a gene designated MMADHC (currently named C2orf25) are responsible for the cblD defect in vitamin B12 metabolism; various mutations are associated with each of the three biochemical phenotypes of the disorder PMID: 18385497

Involvement in disease

Methylmalonic aciduria and homocystinuria type cblD (MMAHCD)

Subcellular Location

Cytoplasm. Mitochondrion.

Tissue Specificity

Widely expressed at high levels.

Database Links

HGNC: 25221

OMIM: 277410

KEGG: hsa:27249

STRING: 9606.ENSP00000301920

UniGene: Hs.5324

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Antibody FAQs What Is an Antibody? How to Choose an Antibody for Scientific Research? The difference between primary antibody and secondary antibod The Meaning of Irregular Antibody Screening Tag Antibodies, Your Good Partner in Protein Research How to Choose the Loading Control Antibodies?

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301-363-4651 (Available 9 a.m. to 5 p.m. CST from Monday to Friday)

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Address

7505 Fannin St., Ste 610, Room 7 (CUBIO Innovation Center), Houston, TX 77054, USA

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