Harlequin verses Merle gene
By Nancy Anderson
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I was contacted by a lady some time ago about a website on the internet that she had visited that had some very disturbing
MIS-information on it about the Harlequin and Merle and since we had already had in-depth discussions about the Harlequin
and Merle genes previously and how it applied to the Harlequin Pinscher of today she thought it was important that the actual
truth be published so that those who were truly interested in the breed and it's genetics could be informed correctly thus my
reason for posting this article.  This article may be linked to by any breeders of HPA Harlequin Pinschers and may be
copied and pasted onto any HPA Harlequin Pinscher breeders website as long as reference is made to it's actual author.
I would also like to try to clear up the confusion as to the "HARLEQUIN PINSCHER" verses the Harlequin gene.
The Harlequin gene is just that - it is a gene that causes a certain visual effect on a dog.
The "HARLEQUIN PINSCHER" is a BREED of dog that was incorrectly named YEARS ago by it's fore-father
breeders who did not understand the difference in Piebald, Ticking, Merle and Harlequin. 
The "HARLEQUIN PINSCHER" of today is the same
as it was then with the exception that while we have been able to replicate the MERLE & Piebald patterns we have not
been fortunate enough to replicate the Harlequin pattern assuming that there was actually the Harlequin gene in the breed
which is now being questioned with the advancement of research.  It is now believed that the EXTREME PIEBALD
gene accompanied by the Ticking gene as seen in the Dalmatian is what occurred in the ole timey Harlequin
Pinscher and not the Harlequin gene at all..
I know of no other clearer or more comprehendible way to state that a Harlequin Pinscher is such by BREED
and while it can be Harlequin in pattern it does not have to be and has never had to be yet there are still
some out there that can not seem to understand this very simple concept.   
For those that no nothing about how the genes work and have previously said that the Harlequin gene was a part
of the Miniature Pinscher and Harlequin Pinscher from days past but the Merle gene was NOT then I can only
regard those as being very misinformed and ignorant as to the Harlequin gene as the Merle gene MUST be present
in order for the Harlequin gene to modify it to achieve the Harlequin pattern.   I ask that you ALL be sure that you
know what your talking about before you go saying things and giving advise that it is quite clear that you know
nothing about.   I also advise everybody to take the talk forums with a grain of salt as far as factual information goes
as they have proved over and over and throughout their existence that those that post there are the ones least likely to
have a clue as to what they are talking about.   Unfortunately that holds true for MANY websites as well.
   2009 was a very productive year in research on the Merle and Harlequin genes and it takes very little effort on anybody's part who
is "truly" interested in the genes to be kept informed of the results of the research but since some apparently have not kept up with
the current research I feel the need to post it here for all to see as the Merle and Harlequin genes are not ones that should be in a
breeding program of some one who does not understand the consequences of IMPROPER breeding. 
Dr. Leigh Ann Clark is responsible for new discoveries associated with the Harlequin gene in 2009.

To date the ONLY breed confirmed to have the actual Harlequin gene itself is the Great Dane.  That doesn't mean that many

breed clubs, breeders and registries don't label their own given pattern as a harlequin, it just means that the gene itself does not exist

in that breed which is very misleading and causes massive confusion.  This would easily confirm that the Harlequin gene was a mutation

of the Merle gene many many many years ago in the Great Dane breed and since the Great Dane has not been the founding breed for

other breed development it remains the only known breed with the Harlequin gene.

Let's start with the genes themselves and what their genetic reference is:
MERLE - a dog with a solid coat that has the Merle dilution gene that dilutes random area's of the coat to a lighter color.
The merle gene is NOT carried thus must be shown to be present and be reproduced (*Dominant)
mm NON-MERLE - the merle dilution gene is not present and is NOT carried therefore Merle is not present in any form.
MM DOUBLE MERLE - A merle dog that has 2 merle genes thus double dosed that turns the already merle diluted spots to white.  25% of double merles experience health issues in the form of deformities, deafness, blindness and or early death.

SEMI-Lethal thus no 2 merles should be bred together for double merle offspring.

Harlequin - a merle dog with a protein modifying gene that changes all merle modified areas to white.
A Harlequin is a merle dog with modified coat pattern.
The Harlequin gene is dominant and requires the merle gene to be present for it to occur.
A Harlequin is HhMm
A Harlequin appears WHITE with multiple random  ONE-colored areas.
hh NON-HARLEQUIN - a dog without the Harlequin modifying gene thus Harlequin is NOT present in any form.
HH DOUBLE HARLEQUIN - LETHAL and there will be no survivors of Double Harlequin.

I will include the Piebald gene in this chart as it does exist and could be mistaken for the gene that is present in Double Merles and Harlequins.

Irish Spotting or Tuxedo - white on legs, tail, stomach, chest, neck (*collar), and possibly on chin and face. 
This gene is designed to make the dog appear to be wearing a tuxedo if it was stood on it's hind legs.
Over the years we have actually separated this gene into three (*3) different ones by appearance only.
Irish - White on toes and parts of the leg, chest and possibly on chin, face and stomach.  No collar markings.
If the IRISH marked dog also had colored "POINTS" it would be called a "TRI".
Tuxedo - White on toes, up the leg, on the chest, stomach, partial to full collar and possibly on chin and face.
PIEBALD or Spotting Gene - Gene responsible for the spotted effect of a dogs coat.  Piebald is a BASE
coat color of WHITE overlaid with colored patches/spots. 
Extreme Piebald - such as seen in the Dalmatian where only ticking is responsible for color.
A Dalmatian is Piebald with ticking and not Harlequin.  The proof is simply that if it were Harlequin then breeding 2
Harlequins over the years would of produced Double Harlequin offspring which would of resulted in the extinction
of the Dalmatian many years ago.
The extreme piebald gene should be bred very cautiously as well as with it often comes health issues as well
such as deafness.



T - dominant produces ticking (in homozygous , produces tiny spots over the whole body)

t - recessive - non ticked (does nothing)

Ticking is still a dominant gene, but "doubling up" on it does not cause any problems.
It is a possibility that dogs who are homozygous for ticking will have heavier ticking that those that are heterozygous.
The ticking gene would go visually unnoticed on a black or brown animal if it was present.


BRINDLE - k^br or K^br - Striped

Dominant needing only 1 copy of the gene to be expressed/visual/produced.

k^br is a non-black brindle or colored brindle - i.e. Red Brindle, Fawn Brindle, Blue Brindle, Brown Brindle.
K^br is a Black brindle thus the brindling would show only on the points if the dog had points.
K^br is often considered to be a masked or phantom brindle and many incorrectly think the dog is a recessive carrier when in fact it is a brindle dog but the brindle can not be seen due to variation genes.
* "MIS-MARK" or "White Marking"
I also feel the need to include the 'white marking' or 'mis-mark" as so many call it as it has become apparent that
there are many who do not understand the reason that this occurs so often in all breeds of dogs.  
This is fact NOT a gene at all but an incompletion of melanocytes due to late
fetal development, colds or other developmental delay in melanocyte completion causing missing pigment.
The chest and tips of toes are the last place pigment cells migrate to which explains the reason for white toes
and chest on so many dogs that are NOT Piebald carriers.  The white chest on Miniature Pinschers is caused
by this occurrence and NOT the Piebald or Harlequin gene as so many would prefer to believe.
This occurrence is often referred to as "TRI" if 3 colors are present such as with "POINT" markers
but it is NOT the Tri gene but with no other gene name to call it we have defaulted to "TRI"..
To add confusion to what I've already posted I want to make it clear that 2 (*TWO) patterns can be present on the
same dog.  I.E. - Merle/Brindle, Merle/Piebald, Brindle/Piebald, Merle/Tri, Merle/Tuxedo, Brindle/Tri, Brindle/Tuxedo.

In essence the Harlequin is also a double patterned dog with it's own Pattern name.




Notice the colored coat with diluted areas of the same color.

Can be any color associated with the breed with diluted areas of color.

In Harlequin Pinschers that would be Red, Black/Tan, Brown/Tan,
Blue/Tan, Blue/Fawn & Fawn/Tan.


Non-Merle (*mm)


Any color and/or pattern that does not represent the Merle dilution area of the coat.

Can be any color that is associated with the breed.

In Harlequin Pinschers that would be Red, Black/Tan, Brown/Tan,
Blue/Tan, Blue/Fawn & Fawn/Tan.


Double Merle (*MM)

Double Merles can only occur with the mating of 2 Merle parents and
is considered to be a no-no by most breeders and some registries.

Double Merles have health issues that are not found in the single merle.

Producing a Merle/Piebald will produce virtually the same visual affect
without the health concerns associated with Double Merle.



This is a picture of a 'true' known Harlequin Great Dane.  Researchers believe that ONLY the
Great Dane has ever had the Harlequin gene.

Visual appearance of WHITE with colored spots.

To-date I have only seen black on white but it is
possible that it could occur in any color associated
with the breed.
Red, Black, Brown, Blue, Blue/Fawn, Fawn
Note:  These could be Extreme Piebald w/ticking   instead of Harlequin.
These pictures are old pictures of the Harlequin Pinscher and advancements in research
suggest that the Harlequin Pinscher may NEVER of had the Harlequin gene but that
of the Extreme Piebald with ticking as seen in the Dalmatian. 


For those interested - Research the Harlequin Great Dane for a known true Harlequin pattern.


Non-Harlequin (*hh)

Any color and/or pattern that does not represent the Harlequin modification area of the coat.

Can be any color and/or pattern that is associated with the breed without the
Harlequin gene modification.


Double Harlequin (*HH)


No pictures available.  All Double Harlequins die during fetal development.





Piebald (*S^p)

WHITE base coat with colored spots.

Can be any color of spots associated with the breed.

Red, Black, Brown, Blue, Blue/Fawn & Fawn.


Extreme Piebald (*S^w)
The Samoyed is an extreme piebald with no other marking genes.
The Dalmatian is an extreme piebald with the
ticking gene causing irregular spots of color.
We should not that Dalmatians are not born
with spots but Harlequins are.
There are several other breeds that have the extreme piebald gene including the Dachshund, Maltese, Boxer ..etc ...
The Dalmatian at one time was thought to be from
the Harlequin gene but advances in research as shown
that to be not the case.
Note:  These could be Extreme Piebald w/ticking   instead of Harlequin.
These pictures are old pictures of the Harlequin Pinscher and advancements in research
suggest that the Harlequin Pinscher may NEVER of had the Harlequin gene but that
of the Extreme Piebald with ticking as seen in the Dalmatian. 


TRI - Mis-Mark
Notice the very small spot of white on her chest.  She is actually a mismarked
from lack of pigment development during fetal development but we refer to
this as Tri anyway to distinguish her from a solid with no white.
This is a "TRI".  Notice she has white up her leg, on her chest and chin
but not around her neck forming a collar.


This is a Merle/Piebald which is a white base coated dog with spots
that also have diluted areas of color. 
The Harlequin gene is not involved with this pattern in any form.



 There is a breeder who proclaims hers that is this very
pattern to be a Harlequin but she is incorrect. 



If you look closely you can see the brindling in all of the
pointed area of this dog.

He is a K^br - black brindle.

I've made this picture larger in the hopes that the
brindle will be visible.

Red brindle

a Red dog with tiger stripes


Brindles are without a doubt the hardest of patterns
to get good pictures of that show the pattern.

Brindle is a pattern and can be on any color dog.

A COLOR should always precede the description of a brindle.

i.e.  Black/Tan Brindle, Red Brindle, Blue/Tan Brindle, Brown/Tan Brindle, Fawn/Tan Brindle.



Can you tell the DIFFERENCE:

New Research leads us to
believe this is an
"Extreme Piebald w/ticking"
(*Great Dane)
You should have no trouble in seeing the difference in a Merle/Piebald from the Harlequin and Extreme Piebald w/ticking.
A Harlequin or an Extreme Piebald w/ticking will NOT have a solid colored head.
Beware of those saying theirs are 'true' Harlequins yet their site is full of misinformation and the pictures they post clearly shows a
dog of Merle/Piebald pattern. 

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