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Huntingtin, dopamine receptors and fukutin


Huntingtin is a protein of 3144 amino-acids and Mr of 350kDa. The defect in Huntington's disease is due to expansion of a poly-glutamine repeat sequence near the N-terminus, which exerts a deleterious, dominant-negative effect on the survival of specific neurons in the brain.
Note: The first 3 mAbs in this list are considered to be the most generally useful when 3 Abs against different epitopes are required. The remainder are for specialist applications only.


Abbreviations: wb = western blot if = immunolocalization ip = immunoprecipitation hu = human mo = mouse xe = xenopus lævis ch = chick dm = drosophila fi = fish (raja clavata) CRX = cross-reacting proteins p = preparation m = mapping a = applications

Name

Clone

Type

Epitope (aa)

Applications

Species
ve (x-ve)

References

Huntingtin 19 mAbs

HDA3E103E10G2a1171-1177wb, ifhu, mo, rb30
HDB4E104E10G11844-2131wb, ifhu, mo, rb30
HDC8A48A4G12703-2911wb, ifhu, mo, rb30
HDA2C112C11G11171-1177wb,hu, mo, rb30
HDA5E85E8G11171-1177wb,hu, mo, rb30
HDA7D27D2G11171-1177wb,hu, mo, rb30
HDA12G1012G10G11171-1177wb, ifhu, mo, rb30
HDA6B126B12G2a997-1276wb, ifhu, mo, rb30
HDA6F56F5G1997-1276wbhu, mo, rb30
HDB5C125C12G2a1844-2131wbhu, mo, rb30
HDB1A11A1G11844-2131wbhu, mo, rb30
HDB7D77D7G2b2081-2086wbhu, mo, rb30
HDB5A125A12G2a2081-2086wbhu, mo, rb30
HDB4F84F8G2a2081-2086wbhu, mo, rb30
HDB4D84D8G2b2081-2086wbhu, mo, rb30
HDB1D101D10G2b2081-2086wbhu, mo, rb30
HDC9B49B4G12703-2911wb, ifhu, mo, rb30
HDC11A311A3G12703-2911wbhu, mo, rb30
HDC8G38G3G12703-2911wbhu, mo, rb30

Dopamine receptors

Dopamine receptors are members of the G protein-coupled receptor (GPCR) family, which initiate their biological function by coupling to GTP regulatory proteins (G proteins). In humans, five dopamine receptor subtypes have been identified. These receptors have been grouped into two subfamilies: the D1-like receptors and the D2-like receptors. Abnormalities in dopamine neurotransmission are implicated in the aetiology of several major neurological and psychiatric disorders including schizophrenia and Parkinsons’s disease. The antibodies available are against the three D2-like receptors, DRD2, DRD3 and DRD4.


Name

Clone

Type

Epitope (aa)

Applications

Species
ve (x-ve)

References

DRD2 08 mAbs

MANDOP 311D7, 129Ig2a2 -10wb, ifhu, rat44
MANDOP 323F1, 121Ig2a2 - 10wb, ifhu, rat44
MANDOP 333B7, 123IgM210 - 218wb, ifhu, rat44
MANDOP 344D10, 85IgM210 - 218wb, ifhu, rat44
MANDOP 359F4, 63IgM210 - 218wb, ifhu, rat44
MANDOP 213B9, 29n. d272 - 282wb, ifhu, rat44
MANDOP 223D9, 117IgM272 - 282wb, ifhu, rat44
MANDOP 412B9, 212IgG1176 - 185wb, ifhu, rat44

Fukutin


Mutations in fukutin also cause Fukuyama-type congenital muscular dystrophy (FCMD: MIM 253800), one of the most prevalent autosomal recessive disorders in the Japanese population. Mutations in fukutin cause abnormal glycosylation of cell surface a-dystroglycan which in turn reduces its laminin-binding activity, but a direct catalytic function for fukutin has not been established.


Abbreviations: wb = western blot if = immunolocalization ip = immunoprecipitation hu = human mo = mouse xe = xenopus lævis ch = chick dm = drosophila fi = fish (raja clavata) CRX = cross-reacting proteins p = preparation m = mapping a = applications

Name

Clone

Type

Epitope (aa)

Applications

Species
ve (x-ve)

References

Fukutin 12 mAbs

MANFU17A2IgG1not mappedwb, ifhu56
MANFU210F9 IgG2aGroup 4 AA243-246 (PHSR) by phage-displaywb, ifhu56
MANFU34E6 IgG2aGroup 4 AA243-246 (PHSR) by phage-displaywb, ifhu56
MANFU43C7 IgG2aGroup 1 AA223-231 (TVDGLEVLI) by phage-displaywb, ifhu56
MANFU57H2 IgG2anot mappedwb, ifhu56
MANFU64H8 IgG2anot mappedwb, ifhu56
MANFU71B5 IgG2bGroup 3 AA180-187(SGNYL - HG) by phage-displaywb, ifhu56
MANFU82F5 IgG2bGroup 3 AA180-187(SGNYL - HG) by phage-displaywb, ifhu56
MANFU94E12 IgG2aGroup 3 AA180-187(SGNYL - HG) by phage-displaywb, ifhu56
MANFU105H10Ig2bGroup 3 AA180-187(SGNYL - HG) by phage-displaywb, ifhu56
MANFU113E4 IgG1Group 2 AA452-461(SEWD – VI - - Y) by phage-displaywb, ifhu56
MANFU126B5 IgG2aGroup 2 AA452-461(SEWD – VI - - Y) by phage-displaywb, ifhu56